Oreoluwa E Olakunle, Rachel S Tobin, Nicole Guynn, Anita Osabutey, Maya Younker, Mahnoor Khan, Marina Mosunjac, Alison F Ward, Modele O Ogunniyi
BACKGROUND Constrictive pericarditis occurs due to chronic pericardial inflammation and adherence of the cardiac pericardial layer. Etiologies include toxins, infection, cardiac surgery, and idiopathic causes. Outside the United States, the most common cause of constrictive pericarditis is tuberculosis (TB). Constrictive pericarditis is the most severe complication of tuberculous pericardial disease. CASE REPORT A 31-year-old man who recently immigrated to the United States presented with a 2-week history of constitutional symptoms, dyspnea, and pleuritic chest pain. Physical examination was pertinent for bilateral lower extremity pitting edema, decreased bilateral breath sounds, and jugular venous distension. Transthoracic echocardiogram revealed a left ventricular ejection fraction of 45%, pericardial thickening, and an exaggerated septal bounce. Right heart catheterization showed discordant and concordant right ventricular pressure tracings. Cardiac magnetic resonance imaging revealed bilateral pleural effusions and circumferential pericardial thickening. Thoracocentesis was significant for an exudative effusion, with elevated adenosine deaminase levels. Subsequent QuantiFERON-TB Gold testing was positive, and he underwent elective pericardiectomy. Pericardial histopathology revealed necrotizing caseating granulomas. He was discharged on a 6-month course of rifampicin, isoniazid, pyrazinamide, and ethambutol therapy, with close multidisciplinary care team outpatient follow-up. CONCLUSIONS This case highlights the importance of a high index of clinical suspicion for tuberculous pericarditis in patients presenting with constitutional and heart failure symptoms and a relevant travel history, to ensure prompt diagnosis and treatment. This case also reflects the importance of coordination of care between cardiology, infectious disease, pathology, and cardiothoracic surgery teams in the management of tuberculous constrictive pericarditis.
{"title":"The Constricted Heart: A 31-Year-Old Man with a Case of Constrictive Tuberculous Pericarditis.","authors":"Oreoluwa E Olakunle, Rachel S Tobin, Nicole Guynn, Anita Osabutey, Maya Younker, Mahnoor Khan, Marina Mosunjac, Alison F Ward, Modele O Ogunniyi","doi":"10.12659/AJCR.944607","DOIUrl":"https://doi.org/10.12659/AJCR.944607","url":null,"abstract":"<p><p>BACKGROUND Constrictive pericarditis occurs due to chronic pericardial inflammation and adherence of the cardiac pericardial layer. Etiologies include toxins, infection, cardiac surgery, and idiopathic causes. Outside the United States, the most common cause of constrictive pericarditis is tuberculosis (TB). Constrictive pericarditis is the most severe complication of tuberculous pericardial disease. CASE REPORT A 31-year-old man who recently immigrated to the United States presented with a 2-week history of constitutional symptoms, dyspnea, and pleuritic chest pain. Physical examination was pertinent for bilateral lower extremity pitting edema, decreased bilateral breath sounds, and jugular venous distension. Transthoracic echocardiogram revealed a left ventricular ejection fraction of 45%, pericardial thickening, and an exaggerated septal bounce. Right heart catheterization showed discordant and concordant right ventricular pressure tracings. Cardiac magnetic resonance imaging revealed bilateral pleural effusions and circumferential pericardial thickening. Thoracocentesis was significant for an exudative effusion, with elevated adenosine deaminase levels. Subsequent QuantiFERON-TB Gold testing was positive, and he underwent elective pericardiectomy. Pericardial histopathology revealed necrotizing caseating granulomas. He was discharged on a 6-month course of rifampicin, isoniazid, pyrazinamide, and ethambutol therapy, with close multidisciplinary care team outpatient follow-up. CONCLUSIONS This case highlights the importance of a high index of clinical suspicion for tuberculous pericarditis in patients presenting with constitutional and heart failure symptoms and a relevant travel history, to ensure prompt diagnosis and treatment. This case also reflects the importance of coordination of care between cardiology, infectious disease, pathology, and cardiothoracic surgery teams in the management of tuberculous constrictive pericarditis.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142308724","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
BACKGROUND A choledochal cyst (CC), or biliary cyst, is a congenital or acquired anomaly of the biliary tree. Pancreas divisum (PD) is a rare congenital anomaly due to incomplete fusion of pancreatic ducts, which can complicate the clinical course of choledochal cysts. This rare combination is a surgical management challenge. This report presents the diagnosis and management of a 23-year-old woman with a combined choledochal cyst and pancreas divisum treated with pancreaticoduodenectomy. CASE REPORT This article presents the case of a 23-year-old woman who presented with severe, stabbing abdominal pain radiating to the back and epigastric tenderness and was diagnosed with pancreatitis. Initial imaging revealed a choledochal cyst, prompting further investigation with ERCP that showed concomitant PD. She was treated via pancreaticoduodenectomy. During the following 9 years, she was hospitalized over 2 dozen times for recurrent pancreatitis. CONCLUSIONS This report presents a complex case of a combined choledochal cyst and pancreas divisum, which was surgically managed by pancreaticoduodenectomy. The association of CC with PD should be suspected in patients with recurrent acute pancreatitis and/or cholangitis with no identifiable cause. Surgical treatment of CC with PD depends on the classification of the CC, and complications can include recurrent pancreatitis, though prognosis is often favorable. The purpose of this manuscript is to emphasize that pancreaticoduodenectomy is unlikely to provide favorable outcomes for CC with PD, especially considering there is evidence that less extensive surgical interventions produce better outcomes.
{"title":"Choledochal Cyst and Pancreas Divisum: A Case Report.","authors":"Peter Tortora, Kushagra Kumar","doi":"10.12659/AJCR.944747","DOIUrl":"https://doi.org/10.12659/AJCR.944747","url":null,"abstract":"<p><p>BACKGROUND A choledochal cyst (CC), or biliary cyst, is a congenital or acquired anomaly of the biliary tree. Pancreas divisum (PD) is a rare congenital anomaly due to incomplete fusion of pancreatic ducts, which can complicate the clinical course of choledochal cysts. This rare combination is a surgical management challenge. This report presents the diagnosis and management of a 23-year-old woman with a combined choledochal cyst and pancreas divisum treated with pancreaticoduodenectomy. CASE REPORT This article presents the case of a 23-year-old woman who presented with severe, stabbing abdominal pain radiating to the back and epigastric tenderness and was diagnosed with pancreatitis. Initial imaging revealed a choledochal cyst, prompting further investigation with ERCP that showed concomitant PD. She was treated via pancreaticoduodenectomy. During the following 9 years, she was hospitalized over 2 dozen times for recurrent pancreatitis. CONCLUSIONS This report presents a complex case of a combined choledochal cyst and pancreas divisum, which was surgically managed by pancreaticoduodenectomy. The association of CC with PD should be suspected in patients with recurrent acute pancreatitis and/or cholangitis with no identifiable cause. Surgical treatment of CC with PD depends on the classification of the CC, and complications can include recurrent pancreatitis, though prognosis is often favorable. The purpose of this manuscript is to emphasize that pancreaticoduodenectomy is unlikely to provide favorable outcomes for CC with PD, especially considering there is evidence that less extensive surgical interventions produce better outcomes.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142297649","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
BACKGROUND Parasitic leiomyoma refers to leiomyomas outside the uterus, with a prevalence of 0.07%. Patients are initially asymptomatic and may later develop abdominal pain and abdominal distension. Parasitic leiomyomas at a trocar site are extremely rare and lack detailed reporting. Here, we report 2 cases of parasitic leiomyoma at trocar sites. CASE REPORT Case 1. The patient was a 47-year-old woman with parasitic leiomyomas at a left trocar site 4 years after laparoscopic total hysterectomy. After being diagnosed with 3 masses on the surface of the sigmoid colon and 2 in the pelvic cavity, the patient underwent laparoscopic removal of a pelvic lesion and 3 lesions on the surface of the colon, combined with excision of abdominal wall masses. The pathology result indicated that the masses at the left trocar site were multiple leiomyomas, the intestinal mass was multiple leiomyomas with abundant cells, and the pelvic mass was fibrous capsule parietal tissue. This patient received 3 months of gonadotropin-releasing hormone agonist (GnRH-a) treatment, and was followed up for 9 months without recurrence. Case 2. The patient was a 50-year-old woman with parasitic leiomyoma at the right trocar site 15 years after laparoscopic removal of the right ovarian cyst. At admission, she underwent transabdominal total hysterectomy, bilateral fallopian tube resection, and abdominal wall lesion resection. The pathology report showed multiple leiomyomas of the uterus, and the cell-rich parasitic leiomyoma at right trocar site with unclear boundary. She received 3 months of GnRH-a treatment, and was followed up for 6 months without recurrence. CONCLUSIONS For patients with a history of laparoscopy, gynecologists should be alert to the occurrence of parasitic leiomyoma.
{"title":"Parasitic Leiomyoma at Laparoscopic Trocar Site: A Report of 2 Cases.","authors":"Bingxin Chen, Zaigui Wu, Fang Tang, Jianhong Zhou, Fei Ruan","doi":"10.12659/AJCR.944951","DOIUrl":"https://doi.org/10.12659/AJCR.944951","url":null,"abstract":"<p><p>BACKGROUND Parasitic leiomyoma refers to leiomyomas outside the uterus, with a prevalence of 0.07%. Patients are initially asymptomatic and may later develop abdominal pain and abdominal distension. Parasitic leiomyomas at a trocar site are extremely rare and lack detailed reporting. Here, we report 2 cases of parasitic leiomyoma at trocar sites. CASE REPORT Case 1. The patient was a 47-year-old woman with parasitic leiomyomas at a left trocar site 4 years after laparoscopic total hysterectomy. After being diagnosed with 3 masses on the surface of the sigmoid colon and 2 in the pelvic cavity, the patient underwent laparoscopic removal of a pelvic lesion and 3 lesions on the surface of the colon, combined with excision of abdominal wall masses. The pathology result indicated that the masses at the left trocar site were multiple leiomyomas, the intestinal mass was multiple leiomyomas with abundant cells, and the pelvic mass was fibrous capsule parietal tissue. This patient received 3 months of gonadotropin-releasing hormone agonist (GnRH-a) treatment, and was followed up for 9 months without recurrence. Case 2. The patient was a 50-year-old woman with parasitic leiomyoma at the right trocar site 15 years after laparoscopic removal of the right ovarian cyst. At admission, she underwent transabdominal total hysterectomy, bilateral fallopian tube resection, and abdominal wall lesion resection. The pathology report showed multiple leiomyomas of the uterus, and the cell-rich parasitic leiomyoma at right trocar site with unclear boundary. She received 3 months of GnRH-a treatment, and was followed up for 6 months without recurrence. CONCLUSIONS For patients with a history of laparoscopy, gynecologists should be alert to the occurrence of parasitic leiomyoma.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142308723","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Joanna Ożga, Elżbieta Mężyk, Wojciech Kmiecik, Wadim Wojciechowski, Zbigniew Żuber
BACKGROUND Chronic nonbacterial osteomyelitis (CNO) is a multifocal autoinflammatory bone disease mainly affecting children and adolescents. Sacroiliitis is an inflammation of the sacroiliac joint, diagnosed with the use of musculoskeletal MRI due to its ability to visualize active inflammatory lesions. Ehlers-Danlos syndrome (EDS) is non-inflammatory hereditary disorder of connective tissue. Here, we report the case of a 17.5-year-old female patient with classical EDS and long-term course of the CNO with coexistence of sacroiliac arthritis. CASE REPORT On admission, a patient with CNO reported pain in the scapula, thoracic spine, shoulders, and iliac region, with morning stiffness present for 5 months. Physical examination revealed knee and elbow joint hyperextension, hypermobility of the phalanges, increased range of motion of the hip joints, and the presence of reticular rash on the face. In the laboratory blood tests, minor leukocytosis was reported. During hospitalization, a whole-body MRI was performed, detecting bone marrow edema in the Th3, Th4, and Th7 vertebral bodies and the head of seventh rib on the left side, as well as bilaterally in the sacroiliac joints. The patient was diagnosed with sacroiliitis and EDS and successfully treated with risedronate sodium, methotrexate with folic acid, sulfasalazine, and meloxicam, achieving CNO remission and reduced severity of axial skeleton pain. CONCLUSIONS The coexistence of these 3 diseases - CNO, sacroiliac arthritis, and EDS - in the same patient is rare and requires interphysician collaboration to determine the correct diagnosis and subsequently arrange multi-speciality therapeutic management to achieve remission.
{"title":"Coexisting Sacroiliac Arthritis and Chronic Nonbacterial Osteomyelitis in an Adolescent with Ehlers-Danlos Syndrome: A Case Report and Treatment Success.","authors":"Joanna Ożga, Elżbieta Mężyk, Wojciech Kmiecik, Wadim Wojciechowski, Zbigniew Żuber","doi":"10.12659/AJCR.943579","DOIUrl":"https://doi.org/10.12659/AJCR.943579","url":null,"abstract":"<p><p>BACKGROUND Chronic nonbacterial osteomyelitis (CNO) is a multifocal autoinflammatory bone disease mainly affecting children and adolescents. Sacroiliitis is an inflammation of the sacroiliac joint, diagnosed with the use of musculoskeletal MRI due to its ability to visualize active inflammatory lesions. Ehlers-Danlos syndrome (EDS) is non-inflammatory hereditary disorder of connective tissue. Here, we report the case of a 17.5-year-old female patient with classical EDS and long-term course of the CNO with coexistence of sacroiliac arthritis. CASE REPORT On admission, a patient with CNO reported pain in the scapula, thoracic spine, shoulders, and iliac region, with morning stiffness present for 5 months. Physical examination revealed knee and elbow joint hyperextension, hypermobility of the phalanges, increased range of motion of the hip joints, and the presence of reticular rash on the face. In the laboratory blood tests, minor leukocytosis was reported. During hospitalization, a whole-body MRI was performed, detecting bone marrow edema in the Th3, Th4, and Th7 vertebral bodies and the head of seventh rib on the left side, as well as bilaterally in the sacroiliac joints. The patient was diagnosed with sacroiliitis and EDS and successfully treated with risedronate sodium, methotrexate with folic acid, sulfasalazine, and meloxicam, achieving CNO remission and reduced severity of axial skeleton pain. CONCLUSIONS The coexistence of these 3 diseases - CNO, sacroiliac arthritis, and EDS - in the same patient is rare and requires interphysician collaboration to determine the correct diagnosis and subsequently arrange multi-speciality therapeutic management to achieve remission.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142297650","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Vivien Sehapovic, Shehzad Muhamed, Hardeep Singh, Louise Jones
BACKGROUND Reversible cerebral vasoconstriction syndrome (RCVS) is a rare disease that is classified as a condition of diffuse cerebral artery constriction. RCVS can be complicated with transient neurological deficits, seizures, ischemic strokes, and hemorrhagic strokes. A thunder-clap headache, described as being the worst headache a patient can experience, is the predominant symptom in RCVS, which contributes to why RCVS is underdiagnosed as an ischemic stroke or migraine. CASE REPORT In this case study, we present a healthy 34-year-old Black woman who presented to the Emergency Department 3 times over a period of 4 days with concerns of severe headaches. In her first Emergency Department visit, she had a normal computed tomography scan of the brain. Her third Emergency Department visit resulted in hospitalization due to seizures, and a computed tomography brain scan done then showed acute intracranial hemorrhaging. The patient ultimately received a diagnosis of RCVS during her hospitalization. CONCLUSIONS RCVS is the most notable mimicker of other similar-presenting vasculopathies, such as primary angiitis of the central nervous system and posterior reversible encephalopathy syndrome. It is important to note that thunder-clap headache, as well as complications such as intracranial hemorrhaging and seizures, can arise not just from other diseases but from RCVS as well; hence, an early diagnosis is critical to avoid complications, especially if initial imaging is negative.
{"title":"A Rare Case of Idiopathic Reversible Cerebral Vasoconstriction Syndrome.","authors":"Vivien Sehapovic, Shehzad Muhamed, Hardeep Singh, Louise Jones","doi":"10.12659/AJCR.944273","DOIUrl":"https://doi.org/10.12659/AJCR.944273","url":null,"abstract":"<p><p>BACKGROUND Reversible cerebral vasoconstriction syndrome (RCVS) is a rare disease that is classified as a condition of diffuse cerebral artery constriction. RCVS can be complicated with transient neurological deficits, seizures, ischemic strokes, and hemorrhagic strokes. A thunder-clap headache, described as being the worst headache a patient can experience, is the predominant symptom in RCVS, which contributes to why RCVS is underdiagnosed as an ischemic stroke or migraine. CASE REPORT In this case study, we present a healthy 34-year-old Black woman who presented to the Emergency Department 3 times over a period of 4 days with concerns of severe headaches. In her first Emergency Department visit, she had a normal computed tomography scan of the brain. Her third Emergency Department visit resulted in hospitalization due to seizures, and a computed tomography brain scan done then showed acute intracranial hemorrhaging. The patient ultimately received a diagnosis of RCVS during her hospitalization. CONCLUSIONS RCVS is the most notable mimicker of other similar-presenting vasculopathies, such as primary angiitis of the central nervous system and posterior reversible encephalopathy syndrome. It is important to note that thunder-clap headache, as well as complications such as intracranial hemorrhaging and seizures, can arise not just from other diseases but from RCVS as well; hence, an early diagnosis is critical to avoid complications, especially if initial imaging is negative.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142297645","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Abid Qureshi, Kinjal Kasbawala, Monica T Santos, Christina Cuoccio, Sumeet Bahl, Ahmar A Butt, Phillip Xiao, Romulo Genato, Luca Milone
BACKGROUND Hypersplenism, the rapid and premature destruction of blood cells, encompasses a triad of splenomegaly, cytopenias (anemia, leukopenia, or thrombocytopenia), and compensatory bone marrow proliferation. Secondary hypersplenism results from non-intrinsic splenic diseases, such as hemoglobinopathies. Sickle cell disease consists of a group of genotypes, where hemoglobin sickle C disease (HbSC) is the inheritance of hemoglobin S with hemoglobin C. Most homozygous genotypes undergo complete auto-splenectomy by age 6 years, whereas those with HbSC disease rarely do. We report a rare case of hypersplenism and massive splenomegaly in an adult with sickle cell disease, the HbSC genotype, requiring splenectomy. CASE REPORT A 41-year-old woman with known splenomegaly initially presented to the general surgery clinic for management of abdominal pain. She was found to have anemia, indicating cytopenia likely from hypersplenism. Consequently, she underwent splenic artery embolization, followed by an exploratory laparotomy and splenectomy, with an unremarkable postoperative course. CONCLUSIONS Acute splenic sequestration crisis can result from hypersplenism, a potentially fatal complication of sickle hemoglobinemia. The continuous cycle of sickled cell entrapment and stasis causes numerous splenic infarctions, forming splenic parenchymal scar tissue which reduces the spleen's size and functionality - the process of auto-splenectomy. Adults rarely experience these crises past adolescence, which are secondary to the scarring and atrophy from premature auto-splenectomy. Our patient's spleen measured 21.1 cm, larger than the average adult's spleen. In our case, adjunctive preoperative splenic artery embolization likely contributed to decreased intraoperative blood loss during splenectomy, mitigating the need for perioperative transfusions.
背景 脾功能亢进是指血细胞的快速和过早破坏,包括脾脏肿大、细胞减少症(贫血、白细胞减少或血小板减少)和代偿性骨髓增生三联征。继发性脾功能亢进源于非内在性脾脏疾病,如血红蛋白病。镰状细胞病由一组基因型组成,其中血红蛋白镰状 C 病(HbSC)是血红蛋白 S 与血红蛋白 C 的遗传。大多数同型基因型患者在 6 岁前会接受完全的自身脾切除术,而那些患有 HbSC 病的患者很少这样做。我们报告了一例罕见的 HbSC 基因型镰状细胞病成人脾功能亢进和巨大脾肿大病例,患者需要进行脾切除术。病例报告 一名 41 岁女性,已知脾脏肿大,最初因腹痛到普外科门诊就诊。她被发现患有贫血,表明可能是脾功能亢进引起的全血细胞减少。因此,她接受了脾动脉栓塞术,随后进行了探查性开腹手术和脾切除术,术后情况无异常。结论 脾功能亢进可导致急性脾疝危象,这是镰状血红蛋白血症的一种潜在致命并发症。成年人过了青春期后很少会出现这种危机,这是由于过早的自身脾切除造成的瘢痕和萎缩引起的继发性危机。我们患者的脾脏大小为 21.1 厘米,比一般成年人的脾脏要大。在我们的病例中,术前辅助性脾动脉栓塞可能有助于减少脾切除术中的术中失血,从而减轻围手术期输血的需要。
{"title":"Uncommon Presentation of Hypersplenism in Adult Sickle Cell Disease Patients: A Rare Case Report.","authors":"Abid Qureshi, Kinjal Kasbawala, Monica T Santos, Christina Cuoccio, Sumeet Bahl, Ahmar A Butt, Phillip Xiao, Romulo Genato, Luca Milone","doi":"10.12659/AJCR.944693","DOIUrl":"https://doi.org/10.12659/AJCR.944693","url":null,"abstract":"<p><p>BACKGROUND Hypersplenism, the rapid and premature destruction of blood cells, encompasses a triad of splenomegaly, cytopenias (anemia, leukopenia, or thrombocytopenia), and compensatory bone marrow proliferation. Secondary hypersplenism results from non-intrinsic splenic diseases, such as hemoglobinopathies. Sickle cell disease consists of a group of genotypes, where hemoglobin sickle C disease (HbSC) is the inheritance of hemoglobin S with hemoglobin C. Most homozygous genotypes undergo complete auto-splenectomy by age 6 years, whereas those with HbSC disease rarely do. We report a rare case of hypersplenism and massive splenomegaly in an adult with sickle cell disease, the HbSC genotype, requiring splenectomy. CASE REPORT A 41-year-old woman with known splenomegaly initially presented to the general surgery clinic for management of abdominal pain. She was found to have anemia, indicating cytopenia likely from hypersplenism. Consequently, she underwent splenic artery embolization, followed by an exploratory laparotomy and splenectomy, with an unremarkable postoperative course. CONCLUSIONS Acute splenic sequestration crisis can result from hypersplenism, a potentially fatal complication of sickle hemoglobinemia. The continuous cycle of sickled cell entrapment and stasis causes numerous splenic infarctions, forming splenic parenchymal scar tissue which reduces the spleen's size and functionality - the process of auto-splenectomy. Adults rarely experience these crises past adolescence, which are secondary to the scarring and atrophy from premature auto-splenectomy. Our patient's spleen measured 21.1 cm, larger than the average adult's spleen. In our case, adjunctive preoperative splenic artery embolization likely contributed to decreased intraoperative blood loss during splenectomy, mitigating the need for perioperative transfusions.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142297658","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Chunchun Chen, Jinhai Zhu, Chao Zhang, Lin Wang, Yulong Li, Mingnan Du
BACKGROUND Occult breast cancer (OBC) is diagnosed when regional or distant metastases are found without evidence of a primary tumor. The low overall incidence is a great challenge for the management strategy of OBC. Aggressive diagnosis and personalized treatment are feasible treatment strategies for OBC. We report the case of an OBC patient who achieved pathological complete response (pCR) after neoadjuvant chemotherapy. CASE REPORT A 43-year-old woman was admitted to the hospital 6 months after detecting a lump in her left axilla, about the size of a quail egg, but not red or swollen, and the lump gradually grew. Mammography, ultrasound, and magnetic resonance imaging showed a visible left axilla lesion but no nodules in bilateral breasts. A core-needle biopsy of the axilla lesion revealed an invasive carcinoma of breast origin. The tumor cells were estrogen receptors (ER)-negative, progesterone receptor (PR)-negative, and HER2-positive (3+) by immunohistochemistry. The patient was finally diagnosed with HER2-positive, hormone receptor-negative occult breast cancer of the left breast, cT0N2M0, stage IIIA. The TCbHP regimen (docetaxel, carboplatin, trastuzumab, and pertuzumab) as neoadjuvant chemotherapy was given. She underwent a modified radical mastectomy, showing a pCR. Subsequent radiotherapy and HER2-targeted therapy were administrated. CONCLUSIONS This case highlights that even aggressive HER2-positive breast cancer can present as an occult primary tumor. Our clinical experience suggests that neoadjuvant chemotherapy followed by modified radical mastectomy can be effective for treating such rare cases. The patient achieved pCR, which can provide a therapeutic strategy for effective treatment of similar OBCs.
{"title":"Pathological Complete Response with Neoadjuvant Trastuzumab, Pertuzumab, and Chemotherapy Followed by Modified Radical Mastectomy in a Patient with HER2-Positive Occult Breast Cancer.","authors":"Chunchun Chen, Jinhai Zhu, Chao Zhang, Lin Wang, Yulong Li, Mingnan Du","doi":"10.12659/AJCR.943936","DOIUrl":"https://doi.org/10.12659/AJCR.943936","url":null,"abstract":"<p><p>BACKGROUND Occult breast cancer (OBC) is diagnosed when regional or distant metastases are found without evidence of a primary tumor. The low overall incidence is a great challenge for the management strategy of OBC. Aggressive diagnosis and personalized treatment are feasible treatment strategies for OBC. We report the case of an OBC patient who achieved pathological complete response (pCR) after neoadjuvant chemotherapy. CASE REPORT A 43-year-old woman was admitted to the hospital 6 months after detecting a lump in her left axilla, about the size of a quail egg, but not red or swollen, and the lump gradually grew. Mammography, ultrasound, and magnetic resonance imaging showed a visible left axilla lesion but no nodules in bilateral breasts. A core-needle biopsy of the axilla lesion revealed an invasive carcinoma of breast origin. The tumor cells were estrogen receptors (ER)-negative, progesterone receptor (PR)-negative, and HER2-positive (3+) by immunohistochemistry. The patient was finally diagnosed with HER2-positive, hormone receptor-negative occult breast cancer of the left breast, cT0N2M0, stage IIIA. The TCbHP regimen (docetaxel, carboplatin, trastuzumab, and pertuzumab) as neoadjuvant chemotherapy was given. She underwent a modified radical mastectomy, showing a pCR. Subsequent radiotherapy and HER2-targeted therapy were administrated. CONCLUSIONS This case highlights that even aggressive HER2-positive breast cancer can present as an occult primary tumor. Our clinical experience suggests that neoadjuvant chemotherapy followed by modified radical mastectomy can be effective for treating such rare cases. The patient achieved pCR, which can provide a therapeutic strategy for effective treatment of similar OBCs.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142297657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Georgios S Papaetis, Ioannis P Kazakos, Pavlos G Constantinou, Victoria K Evagorou, Stylianos A Karvounaris, Konstantinos C Mikellidis
BACKGROUND Epithelial neoplasms are the most common and heterogenous group of ovarian tumors. Approximately 10-15% are primary ovarian mucinous neoplasms. Almost 80% of these consist of benign mucinous neoplasms, while the rest are borderline neoplasms, non-invasive (intraepithelial and intraglandular) carcinomas, and invasive carcinomas. Small ovarian cystadenomas are generally asymptomatic and are mainly found incidentally during an ultrasound examination for another gynecologic disorder. As their size increases, nonspecific symptoms and clinical signs develop as a result of mass effect to adjacent structures or because of tumor torsion. The main clinical symptoms are abdominal and/or pelvic pain, fullness, and discomfort. Large cystadenomas have also been associated with nausea and vomiting, urinary problems, persistent cough, back pain, metrorrhagia, and feminization. CASE REPORT We report a case of a 31-year-old woman with a body mass index of 39 who presented with increasing sacrococcygeal pain and right leg paresthesia over a 2-year period. She was treated for possible musculoskeletal and spine problems. She was finally diagnosed with a large right ovarian mucinous cystadenoma expanding in the sacrococcygeal region. She was successfully treated with complete excision of the tumor and achieved complete remission of all her symptoms. CONCLUSIONS Large ovarian mucinous cystadenomas, which develop in the sacrococcygeal region, can lead to symptoms that mimic musculoskeletal and spine problems. Early diagnosis is of great importance towards the goal of implementing proper therapeutic approaches and achieve complete remission of all clinical symptoms.
{"title":"Musculoskeletal Pain and Right Leg Paresthesia Revealed as Large Ovarian Mucinous Cystadenoma: A Case Report.","authors":"Georgios S Papaetis, Ioannis P Kazakos, Pavlos G Constantinou, Victoria K Evagorou, Stylianos A Karvounaris, Konstantinos C Mikellidis","doi":"10.12659/AJCR.945083","DOIUrl":"https://doi.org/10.12659/AJCR.945083","url":null,"abstract":"<p><p>BACKGROUND Epithelial neoplasms are the most common and heterogenous group of ovarian tumors. Approximately 10-15% are primary ovarian mucinous neoplasms. Almost 80% of these consist of benign mucinous neoplasms, while the rest are borderline neoplasms, non-invasive (intraepithelial and intraglandular) carcinomas, and invasive carcinomas. Small ovarian cystadenomas are generally asymptomatic and are mainly found incidentally during an ultrasound examination for another gynecologic disorder. As their size increases, nonspecific symptoms and clinical signs develop as a result of mass effect to adjacent structures or because of tumor torsion. The main clinical symptoms are abdominal and/or pelvic pain, fullness, and discomfort. Large cystadenomas have also been associated with nausea and vomiting, urinary problems, persistent cough, back pain, metrorrhagia, and feminization. CASE REPORT We report a case of a 31-year-old woman with a body mass index of 39 who presented with increasing sacrococcygeal pain and right leg paresthesia over a 2-year period. She was treated for possible musculoskeletal and spine problems. She was finally diagnosed with a large right ovarian mucinous cystadenoma expanding in the sacrococcygeal region. She was successfully treated with complete excision of the tumor and achieved complete remission of all her symptoms. CONCLUSIONS Large ovarian mucinous cystadenomas, which develop in the sacrococcygeal region, can lead to symptoms that mimic musculoskeletal and spine problems. Early diagnosis is of great importance towards the goal of implementing proper therapeutic approaches and achieve complete remission of all clinical symptoms.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11416133/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142297656","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Dinh N Nguyen, Abid Qureshi, Michael A Salvian, Phillip Xiao, Cynthia Chen
BACKGROUND Amyloidosis results in fibrillar sheets of beta-pleated amorphous congophilic protein deposition in the extracellular space. Breast amyloidosis is a rare entity, with the first case reported in 1973 and only 2 major case series published since. These deposits can have local or systemic manifestations and typically present unilaterally, although bilateral involvement has been described. Some reported cases of amyloidosis have been linked to breast cancer. CASE REPORT The patient was a 60-year-old woman who presented to the breast surgery clinic for evaluation after image-guided biopsy of a right breast lesion. Core needle biopsy under stereotactic guidance demonstrated pathology consistent with nodular deposition of amyloid, associated with calcifications. Microscopic examination revealed extracellular deposition of acellular eosinophilic material in fat, stoma, and blood vessels. Congo red special stain was positive. Amyloid with Congo red special stain showed apple green birefringence under polarized light. Surgical excision under needle localization was performed, with the final surgical pathology report confirming amyloid deposits. CONCLUSIONS Breast amyloidosis can have calcium affinity, create a foreign body-like reaction with giant cell infiltration, and distribute through periductal, perivascular, or intralobar patterns. Some factors that can contribute to an increased risk or are associated with breast amyloidosis are predisposing clinical conditions, including autoimmune disease, various breast cancers, and B-cell lymphomas. Amyloidosis of the breast should be treated either as primary or secondary, based on etiology. Further studies need to be conducted on whether there are specific genetic markers that predispose populations to the development of amyloidosis of the breast.
背景 淀粉样变性导致细胞外空间沉积β褶状无定形嗜充质蛋白的纤维片。乳腺淀粉样变性是一种罕见病,首例病例于 1973 年报道,此后仅发表过两篇重要的系列病例。这些沉积物可有局部或全身表现,通常单侧出现,但也有双侧受累的病例。一些报道的淀粉样变性病例与乳腺癌有关。病例报告 患者是一名 60 岁的女性,在图像引导下对右侧乳房病灶进行活检后到乳腺外科门诊进行评估。在立体定向引导下进行的核心针活检显示病理与淀粉样蛋白结节性沉积一致,并伴有钙化。显微镜检查显示,脂肪、造口和血管中有细胞外嗜酸性物质沉积。刚果红特殊染色呈阳性。淀粉样蛋白经刚果红特殊染色后,在偏振光下呈现苹果绿双折射。在针刺定位下进行了手术切除,最终的手术病理报告证实了淀粉样蛋白沉积。结论 乳腺淀粉样变性可能具有钙亲和性,会产生巨细胞浸润的异物样反应,并通过导管周围、血管周围或囊内模式分布。导致乳腺淀粉样变性风险增加或与乳腺淀粉样变性相关的一些因素是易患的临床症状,包括自身免疫性疾病、各种乳腺癌和 B 细胞淋巴瘤。乳腺淀粉样变性应根据病因作为原发性或继发性治疗。还需要进一步研究是否有特定的遗传标记使人群易患乳腺淀粉样变性。
{"title":"Amyloidosis Found in the Breast: A Case Report.","authors":"Dinh N Nguyen, Abid Qureshi, Michael A Salvian, Phillip Xiao, Cynthia Chen","doi":"10.12659/AJCR.944755","DOIUrl":"https://doi.org/10.12659/AJCR.944755","url":null,"abstract":"<p><p>BACKGROUND Amyloidosis results in fibrillar sheets of beta-pleated amorphous congophilic protein deposition in the extracellular space. Breast amyloidosis is a rare entity, with the first case reported in 1973 and only 2 major case series published since. These deposits can have local or systemic manifestations and typically present unilaterally, although bilateral involvement has been described. Some reported cases of amyloidosis have been linked to breast cancer. CASE REPORT The patient was a 60-year-old woman who presented to the breast surgery clinic for evaluation after image-guided biopsy of a right breast lesion. Core needle biopsy under stereotactic guidance demonstrated pathology consistent with nodular deposition of amyloid, associated with calcifications. Microscopic examination revealed extracellular deposition of acellular eosinophilic material in fat, stoma, and blood vessels. Congo red special stain was positive. Amyloid with Congo red special stain showed apple green birefringence under polarized light. Surgical excision under needle localization was performed, with the final surgical pathology report confirming amyloid deposits. CONCLUSIONS Breast amyloidosis can have calcium affinity, create a foreign body-like reaction with giant cell infiltration, and distribute through periductal, perivascular, or intralobar patterns. Some factors that can contribute to an increased risk or are associated with breast amyloidosis are predisposing clinical conditions, including autoimmune disease, various breast cancers, and B-cell lymphomas. Amyloidosis of the breast should be treated either as primary or secondary, based on etiology. Further studies need to be conducted on whether there are specific genetic markers that predispose populations to the development of amyloidosis of the breast.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11416132/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142297646","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Daniel Paramythiotis, Eleni Karlafti, Krystallenia Siniosoglou, Dimitrios Tsavdaris, Ioanna Abba Deka, Georgia Raptou, Xanthippi G Mavropoulou, Elizabeth Psoma, Stavros Panidis, Antonios Michalopoulos
BACKGROUND Autoimmune pancreatitis (AIP) is identified as an outlier in the clinical practice of chronic pancreatitis caused by autoimmune system dysfunction. AIP is classified into 3 subtypes: AIP type 1 and AIP type 2, which are both sensitive to corticosteroids, and the recently introduced AIP type 3. CASE REPORT We present a case of a patient who presented with painless obstructive jaundice. Computed tomography (CT) revealed hyperdense gallbladder material, further dilatation of intrahepatic bile ducts, and distention of the bile duct (15 mm). Based on the available clinical data, which were strongly compatible with pancreatic cancer, Whipple surgery was selected as the treatment for this case. The consequent histopathological report revealed areas of pancreatic parenchyma with fibrous connective tissue development and dense inflammatory cell infiltration with lymphocytes and plasmacytes, which showcased IgG4 positivity. The clinical results suggested a diagnosis of AIP type 1, and the patient was referred to his treating physician for further treatment of AIP. Preoperative histological examination of the pancreas, along with evaluation of the radiological and serological features, could have aided in determining the diagnosis of AIP type 1 pancreatitis despite the unique abnormality of this particular case. CONCLUSIONS Given the aforementioned conditions, AIP, even as a rare clinical entity, emerges as a canonical ailment and should be considered a viable possibility in clinical practice since it can exclude the patient from an unnecessary surgery.
{"title":"Focal Autoimmune Pancreatitis Morphologically Mimicking Pancreatic Cancer: A Case Report and Literature Review.","authors":"Daniel Paramythiotis, Eleni Karlafti, Krystallenia Siniosoglou, Dimitrios Tsavdaris, Ioanna Abba Deka, Georgia Raptou, Xanthippi G Mavropoulou, Elizabeth Psoma, Stavros Panidis, Antonios Michalopoulos","doi":"10.12659/AJCR.944286","DOIUrl":"https://doi.org/10.12659/AJCR.944286","url":null,"abstract":"<p><p>BACKGROUND Autoimmune pancreatitis (AIP) is identified as an outlier in the clinical practice of chronic pancreatitis caused by autoimmune system dysfunction. AIP is classified into 3 subtypes: AIP type 1 and AIP type 2, which are both sensitive to corticosteroids, and the recently introduced AIP type 3. CASE REPORT We present a case of a patient who presented with painless obstructive jaundice. Computed tomography (CT) revealed hyperdense gallbladder material, further dilatation of intrahepatic bile ducts, and distention of the bile duct (15 mm). Based on the available clinical data, which were strongly compatible with pancreatic cancer, Whipple surgery was selected as the treatment for this case. The consequent histopathological report revealed areas of pancreatic parenchyma with fibrous connective tissue development and dense inflammatory cell infiltration with lymphocytes and plasmacytes, which showcased IgG4 positivity. The clinical results suggested a diagnosis of AIP type 1, and the patient was referred to his treating physician for further treatment of AIP. Preoperative histological examination of the pancreas, along with evaluation of the radiological and serological features, could have aided in determining the diagnosis of AIP type 1 pancreatitis despite the unique abnormality of this particular case. CONCLUSIONS Given the aforementioned conditions, AIP, even as a rare clinical entity, emerges as a canonical ailment and should be considered a viable possibility in clinical practice since it can exclude the patient from an unnecessary surgery.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11416134/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142297652","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}