Introduction: Cancers of unknown primary (CUPs) present a diagnostic challenge as their origin is unidentified at diagnosis. Massive pleural effusion (MPE), indicative of lung metastasis in CUP, categorizes the condition into an unfavorable subset with a poor prognosis. Patients in this subset may exhibit a lower response to specific therapies.
Case presentation: A 62-year-old woman presented with cough, severe dyspnea, and MPE in the left lung. Thoracocentesis was performed, extracting 1,200 mL of hemorrhagic fluid, followed by the placement of an indwelling pleural catheter. The cytological examination of the pleural effusion indicated an adenocarcinoma, with immunohistochemistry revealing positive CK7 and negative CK20, Napsin A, and TTF-1. Additionally, elevated levels of Ca-125 (1,605 U/mL) and Ca 15-3 (242 U/mL) raised suspicion of gynecological malignancy. Thorax and abdominal CT scans, breast and thyroid ultrasounds showed no signs of malignancy, leading to the diagnosis of CUP. The patient's performance status according to the Eastern Cooperative Oncology Group (ECOG) score was 4. Initial carboplatin 5 AUC and paclitaxel 175 mg/m2 administration resulted in improvement in performance status with ECOG score of 1, alleviation of dyspnea, reduction in pleural effusion 1 week after chemotherapy, with minimal effusion observed at 3 weeks, and Ca-125 levels decreased to 33.6 U/mL thereafter.
Discussion: Empiric chemotherapy using carboplatin and paclitaxel is a feasible option for managing CUP with MPE mimicking gynecological malignancies with elevated Ca-125 and Ca 15-3 markers; initiating chemotherapy in poor performance status patients is beneficial with proper clinical judgment.
{"title":"A Case of CUP with Malignant Pleural Effusion: Overcoming Diagnostic and Therapeutic Hurdles with Chemotherapy.","authors":"Andika Putra, Amadisto Gerwindrawan, Eko Budiono, Bambang Purwanto Utomo, Naela Himayati Afifah, Auliya Suluk Brilliant Sumpono, Heru Pradjatmo, Ibnu Purwanto","doi":"10.1159/000540866","DOIUrl":"https://doi.org/10.1159/000540866","url":null,"abstract":"<p><strong>Introduction: </strong>Cancers of unknown primary (CUPs) present a diagnostic challenge as their origin is unidentified at diagnosis. Massive pleural effusion (MPE), indicative of lung metastasis in CUP, categorizes the condition into an unfavorable subset with a poor prognosis. Patients in this subset may exhibit a lower response to specific therapies.</p><p><strong>Case presentation: </strong>A 62-year-old woman presented with cough, severe dyspnea, and MPE in the left lung. Thoracocentesis was performed, extracting 1,200 mL of hemorrhagic fluid, followed by the placement of an indwelling pleural catheter. The cytological examination of the pleural effusion indicated an adenocarcinoma, with immunohistochemistry revealing positive CK7 and negative CK20, Napsin A, and TTF-1. Additionally, elevated levels of Ca-125 (1,605 U/mL) and Ca 15-3 (242 U/mL) raised suspicion of gynecological malignancy. Thorax and abdominal CT scans, breast and thyroid ultrasounds showed no signs of malignancy, leading to the diagnosis of CUP. The patient's performance status according to the Eastern Cooperative Oncology Group (ECOG) score was 4. Initial carboplatin 5 AUC and paclitaxel 175 mg/m<sup>2</sup> administration resulted in improvement in performance status with ECOG score of 1, alleviation of dyspnea, reduction in pleural effusion 1 week after chemotherapy, with minimal effusion observed at 3 weeks, and Ca-125 levels decreased to 33.6 U/mL thereafter.</p><p><strong>Discussion: </strong>Empiric chemotherapy using carboplatin and paclitaxel is a feasible option for managing CUP with MPE mimicking gynecological malignancies with elevated Ca-125 and Ca 15-3 markers; initiating chemotherapy in poor performance status patients is beneficial with proper clinical judgment.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"17 1","pages":"1194-1200"},"PeriodicalIF":0.7,"publicationDate":"2024-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521488/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142543927","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}
Pub Date : 2024-10-18eCollection Date: 2024-01-01DOI: 10.1159/000541550
Maaz Khan Afghan, Areeb Lutfi, Fatima Qadri, Sahrish Khan, Sana Javaid, Brian Michael Currie, Juan Pablo Rocca, Benjamin Samstein, Erika Hissong, Pashtoon Murtaza Kasi
Introduction: Immune thrombocytopenia (ITP) secondary to durvalumab, a programmed cell death ligand 1 inhibitor, is a rare but clinically significant immune-related adverse event. Herein, we present 2 patients with cholangiocarcinoma who developed ITP immediately post-yttrium-90 radioembolization (Y90-RE) while on durvalumab-based systemic therapy. We hypothesize that given the timing, the immunotherapy and the radioembolization combination led to this event. It is not uncommon given the approval of immunotherapy and its role in locoregional therapies, that patients are treated with a combination of systemic immunotherapy and radioembolization or other forms of radiation, thus signifying the importance of potential complications.
Case presentation: Two patients, a 67-year-old female and a 60-year-old man, with biopsy-proven advanced unresectable cholangiocarcinoma, received a combination of systemic therapy with durvalumab, gemcitabine, and cisplatin and subsequently Y90-RE. Both patients developed ITP following in the immediate post-Y90-RE period. All other causes of ITP were comprehensively ruled out and treatment for ITP was initiated in the form of high-dose steroid and intravenous immunoglobulins. Durvalumab was discontinued, and only gemcitabine/cisplatin-based chemotherapy was continued thereafter. Due to recurrence, one of the patients required longer courses of steroids as well as thrombopoietin receptor agonists.
Conclusion: Immunotherapy in the form of durvalumab and now pembrolizumab alongside chemotherapy is an approved first-line standard of care. Furthermore, it is not uncommon for patients to receive Y90-RE to improve patient outcomes. This report highlights the development of ITP in 2 patients who received durvalumab alongside Y90-RE. Awareness of this as a potential immune-mediated event is important to allow for close monitoring of platelet counts and for early intervention/management when this occurs.
{"title":"Durvalumab-Induced Immune Thrombocytopenia in Patients with Advanced Cholangiocarcinoma Undergoing Yttrium-90 Radioembolization.","authors":"Maaz Khan Afghan, Areeb Lutfi, Fatima Qadri, Sahrish Khan, Sana Javaid, Brian Michael Currie, Juan Pablo Rocca, Benjamin Samstein, Erika Hissong, Pashtoon Murtaza Kasi","doi":"10.1159/000541550","DOIUrl":"https://doi.org/10.1159/000541550","url":null,"abstract":"<p><strong>Introduction: </strong>Immune thrombocytopenia (ITP) secondary to durvalumab, a programmed cell death ligand 1 inhibitor, is a rare but clinically significant immune-related adverse event. Herein, we present 2 patients with cholangiocarcinoma who developed ITP immediately post-yttrium-90 radioembolization (Y90-RE) while on durvalumab-based systemic therapy. We hypothesize that given the timing, the immunotherapy and the radioembolization combination led to this event. It is not uncommon given the approval of immunotherapy and its role in locoregional therapies, that patients are treated with a combination of systemic immunotherapy and radioembolization or other forms of radiation, thus signifying the importance of potential complications.</p><p><strong>Case presentation: </strong>Two patients, a 67-year-old female and a 60-year-old man, with biopsy-proven advanced unresectable cholangiocarcinoma, received a combination of systemic therapy with durvalumab, gemcitabine, and cisplatin and subsequently Y90-RE. Both patients developed ITP following in the immediate post-Y90-RE period. All other causes of ITP were comprehensively ruled out and treatment for ITP was initiated in the form of high-dose steroid and intravenous immunoglobulins. Durvalumab was discontinued, and only gemcitabine/cisplatin-based chemotherapy was continued thereafter. Due to recurrence, one of the patients required longer courses of steroids as well as thrombopoietin receptor agonists.</p><p><strong>Conclusion: </strong>Immunotherapy in the form of durvalumab and now pembrolizumab alongside chemotherapy is an approved first-line standard of care. Furthermore, it is not uncommon for patients to receive Y90-RE to improve patient outcomes. This report highlights the development of ITP in 2 patients who received durvalumab alongside Y90-RE. Awareness of this as a potential immune-mediated event is important to allow for close monitoring of platelet counts and for early intervention/management when this occurs.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"17 1","pages":"1183-1193"},"PeriodicalIF":0.7,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521497/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142543937","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}
Introduction: Metastatic breast cancer (MBC) presents an enduring and significant challenge for affected women, requiring sustained commitment over the years.
Case presentation: This paper presents a case of a woman affected by bone and visceral MBC with a very long 20-year survival, excellent quality of life, and high resilience. She is now 51 years old and underwent quadrantectomy for breast cancer in 2005, and in 2013, she developed a recurrence with bone and liver metastases. Despite the widespread stage of the disease with visceral compromise, the patient was treated with a multidisciplinary approach that included surgery, chemotherapy, radiotherapy, hormone therapy, bone target agents, metabolic radiotherapy, and ozone therapy for medication-related osteonecrosis of the jaw. Multidisciplinary management results in a complete clinical and metabolic response to treatment in a visceral metastatic setting.
Conclusion: This report supports the possibility of achieving unusual survival outcomes in patients with MBC. This study also highlights the importance of resilience in breast cancer patients who continue to manage their disease and pursue treatment for over 2 decades. Understanding these resilience factors can improve clinical practice and support patients' long-term care.
{"title":"Surviving Twenty Years to Bone and Liver Metastatic Breast Cancer: A Case Reported by Treating Oncologists and the Patient Herself.","authors":"Maria Rosaria Valerio, Vittorio Gebbia, Dario Piazza, Giuseppina Campisi, Eleonora D'Agati, Monica Bazzano","doi":"10.1159/000541391","DOIUrl":"https://doi.org/10.1159/000541391","url":null,"abstract":"<p><strong>Introduction: </strong>Metastatic breast cancer (MBC) presents an enduring and significant challenge for affected women, requiring sustained commitment over the years.</p><p><strong>Case presentation: </strong>This paper presents a case of a woman affected by bone and visceral MBC with a very long 20-year survival, excellent quality of life, and high resilience. She is now 51 years old and underwent quadrantectomy for breast cancer in 2005, and in 2013, she developed a recurrence with bone and liver metastases. Despite the widespread stage of the disease with visceral compromise, the patient was treated with a multidisciplinary approach that included surgery, chemotherapy, radiotherapy, hormone therapy, bone target agents, metabolic radiotherapy, and ozone therapy for medication-related osteonecrosis of the jaw. Multidisciplinary management results in a complete clinical and metabolic response to treatment in a visceral metastatic setting.</p><p><strong>Conclusion: </strong>This report supports the possibility of achieving unusual survival outcomes in patients with MBC. This study also highlights the importance of resilience in breast cancer patients who continue to manage their disease and pursue treatment for over 2 decades. Understanding these resilience factors can improve clinical practice and support patients' long-term care.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"17 1","pages":"1174-1182"},"PeriodicalIF":0.7,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521530/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142543985","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}
Pub Date : 2024-10-16eCollection Date: 2024-01-01DOI: 10.1159/000540262
Malak Chahid, Hanae El Gouach, Mohamed Reda Cherkaoui Jaouad, Meriem Damou, Mohammed Sqalli Houssaini, Fadila Kouhen
Introduction: Lung cancer management in patients with pacemakers presents unique challenges. This report examines the utilization of stereotactic body radiation therapy (SBRT) in such a patient population.
Case presentation: A 75-year-old former smoker with a dual-chamber pacemaker presented with inoperable lung adenocarcinoma. SBRT (48 Gy in 4 fractions) was chosen following multidisciplinary consultation and thorough pretreatment evaluation by a rhythmologist to assess pacemaker integrity. Continuous cardiac monitoring during SBRT detected no arrhythmias. Adjuvant therapy consisted of radiotherapy alone due to the patient's health status and limited evidence supporting chemotherapy in this context. At the 18-month follow-up, no cancer recurrence was observed, and regular device checks confirmed pacemaker integrity.
Conclusion: This case demonstrates the successful management of inoperable lung adenocarcinoma with SBRT in a patient with a pacemaker. It underscores the significance of interdisciplinary cooperation and careful patient assessment to optimize treatment outcomes in this challenging clinical scenario.
{"title":"Confronting Complexity: Stereotactic Body Radiation Therapy for Localized Lung Cancer with a Pacemaker.","authors":"Malak Chahid, Hanae El Gouach, Mohamed Reda Cherkaoui Jaouad, Meriem Damou, Mohammed Sqalli Houssaini, Fadila Kouhen","doi":"10.1159/000540262","DOIUrl":"https://doi.org/10.1159/000540262","url":null,"abstract":"<p><strong>Introduction: </strong>Lung cancer management in patients with pacemakers presents unique challenges. This report examines the utilization of stereotactic body radiation therapy (SBRT) in such a patient population.</p><p><strong>Case presentation: </strong>A 75-year-old former smoker with a dual-chamber pacemaker presented with inoperable lung adenocarcinoma. SBRT (48 Gy in 4 fractions) was chosen following multidisciplinary consultation and thorough pretreatment evaluation by a rhythmologist to assess pacemaker integrity. Continuous cardiac monitoring during SBRT detected no arrhythmias. Adjuvant therapy consisted of radiotherapy alone due to the patient's health status and limited evidence supporting chemotherapy in this context. At the 18-month follow-up, no cancer recurrence was observed, and regular device checks confirmed pacemaker integrity.</p><p><strong>Conclusion: </strong>This case demonstrates the successful management of inoperable lung adenocarcinoma with SBRT in a patient with a pacemaker. It underscores the significance of interdisciplinary cooperation and careful patient assessment to optimize treatment outcomes in this challenging clinical scenario.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"17 1","pages":"1166-1173"},"PeriodicalIF":0.7,"publicationDate":"2024-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521424/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142543934","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}
Introduction: Inflammatory breast cancer is a rare and aggressive subtype, with high breast cancer mortality. Compared to noninflammatory breast cancer, even after treatment and response to standard-of-care breast cancer chemotherapy, it has a high propensity for lymph node involvement, high rates of distant metastasis, and shorter survival. The immune checkpoint inhibitor, pembrolizumab, in combination with chemotherapy is now approved for early triple negative breast cancer (TNBC) and for advanced disease if positive for the programmed cell death ligand 1 protein (PD-L1). The response and survival of metastatic inflammatory TNBC to immunotherapy is largely unreported and we present a case of a young woman with metastatic triple negative inflammatory breast cancer, treated with pembrolizumab, carboplatin, and paclitaxel.
Case presentation: A 46-year-old female presented with de novo metastatic inflammatory TNBC with metastasis to lymph nodes, lung, and bones. She was treated with pembrolizumab, carboplatin, and paclitaxel leading to rapid and complete radiographic response. The response was however short lived, and the patient presented with diffuse disease progression in the lungs with pleural effusions, causing death from respiratory distress.
Conclusion: Treatment for metastatic triple negative inflammatory breast cancer mirrors treatment of metastatic TNBC. In PD-L1 positive disease, treatment with chemotherapy and pembrolizumab is first line and in this case led to robust but short-lived response. Inflammatory breast cancer remains a poorly understood breast cancer subtype, and even in the presence of good treatment response, prognosis and survival remain poor. Further studies are warranted to better understand and treat the disease.
{"title":"Inflammatory Breast Cancer and Transient Complete Radiographic Response to Chemoimmunotherapy: A Case Report.","authors":"Adina Amin, Sasis Sirikanjanapong, Amardeep Kalsi, Evelyn Taiwo","doi":"10.1159/000541314","DOIUrl":"https://doi.org/10.1159/000541314","url":null,"abstract":"<p><strong>Introduction: </strong>Inflammatory breast cancer is a rare and aggressive subtype, with high breast cancer mortality. Compared to noninflammatory breast cancer, even after treatment and response to standard-of-care breast cancer chemotherapy, it has a high propensity for lymph node involvement, high rates of distant metastasis, and shorter survival. The immune checkpoint inhibitor, pembrolizumab, in combination with chemotherapy is now approved for early triple negative breast cancer (TNBC) and for advanced disease if positive for the programmed cell death ligand 1 protein (PD-L1). The response and survival of metastatic inflammatory TNBC to immunotherapy is largely unreported and we present a case of a young woman with metastatic triple negative inflammatory breast cancer, treated with pembrolizumab, carboplatin, and paclitaxel.</p><p><strong>Case presentation: </strong>A 46-year-old female presented with de novo metastatic inflammatory TNBC with metastasis to lymph nodes, lung, and bones. She was treated with pembrolizumab, carboplatin, and paclitaxel leading to rapid and complete radiographic response. The response was however short lived, and the patient presented with diffuse disease progression in the lungs with pleural effusions, causing death from respiratory distress.</p><p><strong>Conclusion: </strong>Treatment for metastatic triple negative inflammatory breast cancer mirrors treatment of metastatic TNBC. In PD-L1 positive disease, treatment with chemotherapy and pembrolizumab is first line and in this case led to robust but short-lived response. Inflammatory breast cancer remains a poorly understood breast cancer subtype, and even in the presence of good treatment response, prognosis and survival remain poor. Further studies are warranted to better understand and treat the disease.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"17 1","pages":"1157-1165"},"PeriodicalIF":0.7,"publicationDate":"2024-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521518/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142543960","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}
Introduction: Treatment with topotecan is standard-of-care therapy for relapsed small-cell lung cancer (SCLC). Both oral and intravenous administrations of topotecan have been extensively researched and are found to be equally effective with less adverse events in the oral group.
Case presentation: We report a case of a patient with SCLC, who had previously received oral topotecan, with radiological stable disease with no changes in tumor or metastasis diameter size after two administrations. Subsequently, this patient received intravenous topotecan instead of oral due to supply difficulties. After one administration of intravenous topotecan, we saw significant disease regression.
Conclusion: This is to our knowledge the first reported case of better response of intravenous topotecan than oral topotecan. Multiple extrinsic (e.g., food, medication) factors were investigated but could not deliver an explanation.
{"title":"Is Intravenous and Oral Topotecan in Small-Cell Lung Cancer Truly Equal? A Case Report.","authors":"Davien Deraedt, Saartje Verfaillie, Jokke Wynants, Kristof Cuppens","doi":"10.1159/000540861","DOIUrl":"https://doi.org/10.1159/000540861","url":null,"abstract":"<p><strong>Introduction: </strong>Treatment with topotecan is standard-of-care therapy for relapsed small-cell lung cancer (SCLC). Both oral and intravenous administrations of topotecan have been extensively researched and are found to be equally effective with less adverse events in the oral group.</p><p><strong>Case presentation: </strong>We report a case of a patient with SCLC, who had previously received oral topotecan, with radiological stable disease with no changes in tumor or metastasis diameter size after two administrations. Subsequently, this patient received intravenous topotecan instead of oral due to supply difficulties. After one administration of intravenous topotecan, we saw significant disease regression.</p><p><strong>Conclusion: </strong>This is to our knowledge the first reported case of better response of intravenous topotecan than oral topotecan. Multiple extrinsic (e.g., food, medication) factors were investigated but could not deliver an explanation.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"17 1","pages":"1140-1145"},"PeriodicalIF":0.7,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521484/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142543961","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}
Pub Date : 2024-10-14eCollection Date: 2024-01-01DOI: 10.1159/000540707
Michel Meyers, Ahmad Awada, Ioannis Karfis, Daphné t'Kint de Roodenbeke, Hugo Couvert, Charlotte Hanssens, Alain Hendlisz, Natacha Driessens
Introduction: Neuroendocrine neoplasms encompass well-differentiated tumors (NETs) and poorly differentiated carcinomas (neuroendocrine carcinomas [NECs]), which are distinguished by their clinical behavior and molecular characteristics. They can cause paraneoplastic syndromes, such as ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (CS), necessitating prompt recognition and management due to severe hypercortisolism.
Case presentation: A 66-year-old patient with a 3-year history of metastatic mixed neuroendocrine-non-neuroendocrine neoplasm with a NEC and adenocarcinoma component originating from the vulva presented to the emergency department with dyspnea and fatigue. Upon clinical examination, we found widespread hyperpigmentation, a moon-face appearance, hirsutism, buffalo hump, and muscle atrophy. Laboratory investigations revealed severe hypokalemia (2.3 mmol/L), elevated serum cortisol (1,726 nmol/L) and ACTH (194 ng/L) levels. Urinary free cortisol measurement was 21-fold the upper limit of the reference range (3,614.0 nmol/24 h), and cortisol concentration did not decrease after 1mg-dexamethasone suppression test (1,812 nmol/L for an expected value <50 nmol/L), confirming the ACTH-dependent CS. Thoracoabdominal computed tomography (CT) scan demonstrated progressive neoplastic disease in the liver, kidney, lymph nodes, peritoneum, and lungs. Brain magnetic resonance imaging indicated multifocal metastatic infiltration but no evidence of pituitary adenoma. Interestingly, despite a previously negative 68Ga-DOTATATE positron emission tomography (PET)/CT performed 1 year prior, there was moderate somatostatin receptor (SSTR) expression in lymphatic, pulmonary, peritoneal, and bone tissues, suggesting the presence of a component with redifferentiation and re-expression of the SSTR. After the workup, the patient was admitted to a supportive care facility. Hypercortisolism symptoms were effectively managed with an adrenal enzyme inhibitor (ketoconazole) in combination with somatostatin analogs. Unfortunately, the patient was too frail to benefit from peptide receptor radionuclide therapy (PRRT).
Conclusion: This redifferentiation phenomenon in neuroendocrine tumors should be further investigated as patients might be, under certain conditions, eligible for PRRT. Therefore, we suggest that newly occurring paraneoplastic syndromes in patients with NEC should always be evaluated using 68Ga-DOTATATE PET/CT.
{"title":"Ectopic ACTH-Dependent Cushing's Syndrome Emerging at a Late Stage of a Mixed Histology Neuroendocrine Neoplasm: A Case Report.","authors":"Michel Meyers, Ahmad Awada, Ioannis Karfis, Daphné t'Kint de Roodenbeke, Hugo Couvert, Charlotte Hanssens, Alain Hendlisz, Natacha Driessens","doi":"10.1159/000540707","DOIUrl":"10.1159/000540707","url":null,"abstract":"<p><strong>Introduction: </strong>Neuroendocrine neoplasms encompass well-differentiated tumors (NETs) and poorly differentiated carcinomas (neuroendocrine carcinomas [NECs]), which are distinguished by their clinical behavior and molecular characteristics. They can cause paraneoplastic syndromes, such as ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (CS), necessitating prompt recognition and management due to severe hypercortisolism.</p><p><strong>Case presentation: </strong>A 66-year-old patient with a 3-year history of metastatic mixed neuroendocrine-non-neuroendocrine neoplasm with a NEC and adenocarcinoma component originating from the vulva presented to the emergency department with dyspnea and fatigue. Upon clinical examination, we found widespread hyperpigmentation, a moon-face appearance, hirsutism, buffalo hump, and muscle atrophy. Laboratory investigations revealed severe hypokalemia (2.3 mmol/L), elevated serum cortisol (1,726 nmol/L) and ACTH (194 ng/L) levels. Urinary free cortisol measurement was 21-fold the upper limit of the reference range (3,614.0 nmol/24 h), and cortisol concentration did not decrease after 1mg-dexamethasone suppression test (1,812 nmol/L for an expected value <50 nmol/L), confirming the ACTH-dependent CS. Thoracoabdominal computed tomography (CT) scan demonstrated progressive neoplastic disease in the liver, kidney, lymph nodes, peritoneum, and lungs. Brain magnetic resonance imaging indicated multifocal metastatic infiltration but no evidence of pituitary adenoma. Interestingly, despite a previously negative <sup>68</sup>Ga-DOTATATE positron emission tomography (PET)/CT performed 1 year prior, there was moderate somatostatin receptor (SSTR) expression in lymphatic, pulmonary, peritoneal, and bone tissues, suggesting the presence of a component with redifferentiation and re-expression of the SSTR. After the workup, the patient was admitted to a supportive care facility. Hypercortisolism symptoms were effectively managed with an adrenal enzyme inhibitor (ketoconazole) in combination with somatostatin analogs. Unfortunately, the patient was too frail to benefit from peptide receptor radionuclide therapy (PRRT).</p><p><strong>Conclusion: </strong>This redifferentiation phenomenon in neuroendocrine tumors should be further investigated as patients might be, under certain conditions, eligible for PRRT. Therefore, we suggest that newly occurring paraneoplastic syndromes in patients with NEC should always be evaluated using <sup>68</sup>Ga-DOTATATE PET/CT.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"17 1","pages":"1146-1156"},"PeriodicalIF":0.7,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521454/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142543955","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}
Pub Date : 2024-10-09eCollection Date: 2024-01-01DOI: 10.1159/000541329
Robert Bränström, Jan Zedenius, Ivan Shabo, Magnus Lindskog, C Christofer Juhlin
Introduction: Clear cell renal cell carcinoma (ccRCC), the most common type of kidney cancer in adults, presents significant challenges owing to its resistance to conventional therapies. Standard treatment primarily revolves around surgical methods, particularly nephrectomy, which is critical for managing localized diseases. Despite recent advancements, the metastatic potential of ccRCC necessitates ongoing vigilance in postoperative monitoring to manage and detect disease recurrence. Recent shifts in treatment paradigms, especially with the integration of molecular patterns in ccRCC, have enabled the development of targeted therapies. Immune checkpoint and tyrosine kinase inhibitors (TKIs) have become central to managing metastatic ccRCC, offering new hope through improved survival outcomes. Recent studies have corroborated this by demonstrating the benefits of combining these therapies.
Case presentation: This report discusses a case study of a patient with high-grade ccRCC and thyroid metastases initially deemed non-resectable. The combination of immunotherapy and TKIs reduced tumor size, transforming the thyroid metastasis to a resectable state.
Conclusion: This case highlights significant advancements in treatment approaches and the critical in the management of ccRCC, underscoring the necessity for continuous adaptation of clinical practices to incorporate new therapeutic developments.
{"title":"Immunotherapy and Tyrosine Kinase Inhibitor as a Bridge to Surgery for Clear Cell Renal Cell Carcinoma Metastases to the Thyroid: A Case Report and Literature Review.","authors":"Robert Bränström, Jan Zedenius, Ivan Shabo, Magnus Lindskog, C Christofer Juhlin","doi":"10.1159/000541329","DOIUrl":"https://doi.org/10.1159/000541329","url":null,"abstract":"<p><strong>Introduction: </strong>Clear cell renal cell carcinoma (ccRCC), the most common type of kidney cancer in adults, presents significant challenges owing to its resistance to conventional therapies. Standard treatment primarily revolves around surgical methods, particularly nephrectomy, which is critical for managing localized diseases. Despite recent advancements, the metastatic potential of ccRCC necessitates ongoing vigilance in postoperative monitoring to manage and detect disease recurrence. Recent shifts in treatment paradigms, especially with the integration of molecular patterns in ccRCC, have enabled the development of targeted therapies. Immune checkpoint and tyrosine kinase inhibitors (TKIs) have become central to managing metastatic ccRCC, offering new hope through improved survival outcomes. Recent studies have corroborated this by demonstrating the benefits of combining these therapies.</p><p><strong>Case presentation: </strong>This report discusses a case study of a patient with high-grade ccRCC and thyroid metastases initially deemed non-resectable. The combination of immunotherapy and TKIs reduced tumor size, transforming the thyroid metastasis to a resectable state.</p><p><strong>Conclusion: </strong>This case highlights significant advancements in treatment approaches and the critical in the management of ccRCC, underscoring the necessity for continuous adaptation of clinical practices to incorporate new therapeutic developments.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"17 1","pages":"1124-1130"},"PeriodicalIF":0.7,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521524/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142543959","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}
Introduction: There have been only a few cases showing the efficacy of pembrolizumab on granulocyte-colony-stimulating factor (G-CSF)-producing non-small-cell lung cancer (NSCLC) with high programmed cell death ligand 1 (PD-L1) expression. Herein, we report the first case showing the efficacy of pembrolizumab for G-CSF-producing NSCLC with high PD-L1 expression, although the patient had factors indicative of poor pembrolizumab efficacy, such as poor performance status (PS) due to the tumor-induced inflammation and corticosteroids administration.
Case presentation: A 77-year-old woman was diagnosed with G-CSF-producing NSCLC-not otherwise specified, classified as clinical stage IVB, T2N3M1c. She had fever and her PS was 3, and her C-reactive protein (CRP) was 6.47 mg/dL due to inflammation by a G-CSF-producing tumor. Thus, we initiated the administration of dexamethasone (3.3 mg/day). Her fever abated the next day, and CRP dropped to 3.22 mg/dL after 4 days. Driver mutations were negative, and PD-L1, tumor proportion score, was highly expressed at 100%. Thus, pembrolizumab was started. Subsequently, the white blood cell count decreased, and the tumor shrank, indicating a partial response. After three cycles of pembrolizumab therapy, the anorexia improved, and she was discharged. The patient developed sclerosing cholangitis after discharge. Therefore, the pembrolizumab treatment was discontinued. The primary lesion was enlarged, indicating progressive disease. However, the patient and her family did not want additional treatment. Finally, her progression-free survival and overall survival were 6 and 7 months, respectively.
Conclusion: Pembrolizumab may be effective against G-CSF-producing NSCLC with high PD-L1 expression. Corticosteroids seemed to inhibit inflammation induced by the tumor, and exert the efficacy of pembrolizumab.
{"title":"A Case of Granulocyte-Colony-Stimulating Factor-Producing Non-Small Cell Lung Cancer under Steroid Treatment and with Poor Performance Status That Responded to Pembrolizumab.","authors":"Hiroki Egusa, Takeshi Masuda, Kakuhiro Yamaguchi, Shinjiro Sakamoto, Yasushi Horimasu, Kei Kushitani, Taku Nakashima, Hiroshi Iwamoto, Hironobu Hamada, Noboru Hattori","doi":"10.1159/000540935","DOIUrl":"https://doi.org/10.1159/000540935","url":null,"abstract":"<p><strong>Introduction: </strong>There have been only a few cases showing the efficacy of pembrolizumab on granulocyte-colony<i>-</i>stimulating factor (G-CSF)-producing non-small-cell lung cancer (NSCLC) with high programmed cell death ligand 1 (PD-L1) expression. Herein, we report the first case showing the efficacy of pembrolizumab for G-CSF-producing NSCLC with high PD-L1 expression, although the patient had factors indicative of poor pembrolizumab efficacy, such as poor performance status (PS) due to the tumor-induced inflammation and corticosteroids administration.</p><p><strong>Case presentation: </strong>A 77-year-old woman was diagnosed with G-CSF-producing NSCLC-not otherwise specified, classified as clinical stage IVB, T2N3M1c. She had fever and her PS was 3, and her C-reactive protein (CRP) was 6.47 mg/dL due to inflammation by a G-CSF-producing tumor. Thus, we initiated the administration of dexamethasone (3.3 mg/day). Her fever abated the next day, and CRP dropped to 3.22 mg/dL after 4 days. Driver mutations were negative, and PD-L1, tumor proportion score, was highly expressed at 100%. Thus, pembrolizumab was started. Subsequently, the white blood cell count decreased, and the tumor shrank, indicating a partial response. After three cycles of pembrolizumab therapy, the anorexia improved, and she was discharged. The patient developed sclerosing cholangitis after discharge. Therefore, the pembrolizumab treatment was discontinued. The primary lesion was enlarged, indicating progressive disease. However, the patient and her family did not want additional treatment. Finally, her progression-free survival and overall survival were 6 and 7 months, respectively.</p><p><strong>Conclusion: </strong>Pembrolizumab may be effective against G-CSF-producing NSCLC with high PD-L1 expression. Corticosteroids seemed to inhibit inflammation induced by the tumor, and exert the efficacy of pembrolizumab.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"17 1","pages":"1115-1123"},"PeriodicalIF":0.7,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521490/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142543928","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}
Pub Date : 2024-10-09eCollection Date: 2024-01-01DOI: 10.1159/000541341
Nils A Eide, Agate Noer, Henrik Jespersen, Peter Jebsen, Jürgen Geisler
Introduction: This report presents a case of an exceptionally delayed distant recurrence of a choroidal melanoma, occurring 4 decades after the enucleation of the affected eye.
Case presentation: In 1977, a 29-year-old man underwent enucleation for a choroidal melanoma. At the age of 68 years, he was diagnosed with advanced prostate cancer. Although the metastatic prostate cancer responded to treatment, a persistent lung lesion warranted further examination. A lung biopsy, somewhat surprisingly, confirmed the presence of melanoma metastasis, 4 decades after the enucleation. The cells were positive for Melan-A, while no BRAF mutation was identified. Two years later, new lesions appeared in the liver, and CT showed progression with multiple new sites. A liver biopsy revealed again melanoma recurrence, and its choroidal origin was verified by the presence of a GNA11 mutation. The patient underwent radiation therapy for the lung and liver lesions, followed by immunotherapy. However, the patient died 11 months after the recurrence in the liver. In this case report, the micrometastatic melanoma cells appear to have remained dormant for an extended period, before the patient's treatment in 1977, but the reason for the late reactivation from the dormant state remains unclear.
Conclusion: The recurrence of a choroidal melanoma is substantiated by the histopathological and molecular analyses, including the finding of a GNA11 mutation. This case exemplifies a remarkably delayed distant recurrence of a choroidal melanoma, which manifested clinically 40 years following enucleation.
{"title":"Delayed Distant Recurrence of a Uveal Melanoma 4 Decades after Enucleation.","authors":"Nils A Eide, Agate Noer, Henrik Jespersen, Peter Jebsen, Jürgen Geisler","doi":"10.1159/000541341","DOIUrl":"https://doi.org/10.1159/000541341","url":null,"abstract":"<p><strong>Introduction: </strong>This report presents a case of an exceptionally delayed distant recurrence of a choroidal melanoma, occurring 4 decades after the enucleation of the affected eye.</p><p><strong>Case presentation: </strong>In 1977, a 29-year-old man underwent enucleation for a choroidal melanoma. At the age of 68 years, he was diagnosed with advanced prostate cancer. Although the metastatic prostate cancer responded to treatment, a persistent lung lesion warranted further examination. A lung biopsy, somewhat surprisingly, confirmed the presence of melanoma metastasis, 4 decades after the enucleation. The cells were positive for Melan-A, while no <i>BRAF</i> mutation was identified. Two years later, new lesions appeared in the liver, and CT showed progression with multiple new sites. A liver biopsy revealed again melanoma recurrence, and its choroidal origin was verified by the presence of a <i>GNA11</i> mutation. The patient underwent radiation therapy for the lung and liver lesions, followed by immunotherapy. However, the patient died 11 months after the recurrence in the liver. In this case report, the micrometastatic melanoma cells appear to have remained dormant for an extended period, before the patient's treatment in 1977, but the reason for the late reactivation from the dormant state remains unclear.</p><p><strong>Conclusion: </strong>The recurrence of a choroidal melanoma is substantiated by the histopathological and molecular analyses, including the finding of a <i>GNA11</i> mutation. This case exemplifies a remarkably delayed distant recurrence of a choroidal melanoma, which manifested clinically 40 years following enucleation.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"17 1","pages":"1131-1139"},"PeriodicalIF":0.7,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521504/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142543936","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}