Pub Date : 2022-10-18eCollection Date: 2022-01-01DOI: 10.1177/20363613221135987
Safia Yahiaoui, Asma Ghorbel, Khadija Ben Zid, Semia Zarraa, Lina Kchaou, FidaNoubigh, Wael Kaabia, Fadoua Bouguerra, Alia Mousli, Rim Abidi, Amani Yousfi, Boujelbene Nadia, Chiraz Nasr
Langerhans histiocytosis or Langerhans cell histiocytosis (LCH) is a rare benign pathology representing less than 1% of orbital tumors. It can cause either localized or generalized lesions, leading to the destruction of hard and soft tissues. Eosinophilic granuloma is the most benign form and the predominant clinical presentation of LCH. We report a case of eosinophilic granuloma with orbital involvement in an 18-year-old male patient. Orbital radiotherapy was initially planned, but finally it was not performed due to a spontaneous regression of the lesion after the incisional biopsy. The presented case supports an expectant attitude given the possibility of a spontaneous regression after the biopsy, especially in small lesions. However, long-term follow-up is essential given the risk of recurrence.
{"title":"Spontaneous regression of an orbital Langerhans cell histiocytosis after biopsy: A case report. Spontaneous regression of an orbital Langerhans cell histiocytosis.","authors":"Safia Yahiaoui, Asma Ghorbel, Khadija Ben Zid, Semia Zarraa, Lina Kchaou, FidaNoubigh, Wael Kaabia, Fadoua Bouguerra, Alia Mousli, Rim Abidi, Amani Yousfi, Boujelbene Nadia, Chiraz Nasr","doi":"10.1177/20363613221135987","DOIUrl":"https://doi.org/10.1177/20363613221135987","url":null,"abstract":"<p><p>Langerhans histiocytosis or Langerhans cell histiocytosis (LCH) is a rare benign pathology representing less than 1% of orbital tumors. It can cause either localized or generalized lesions, leading to the destruction of hard and soft tissues. Eosinophilic granuloma is the most benign form and the predominant clinical presentation of LCH. We report a case of eosinophilic granuloma with orbital involvement in an 18-year-old male patient. Orbital radiotherapy was initially planned, but finally it was not performed due to a spontaneous regression of the lesion after the incisional biopsy. The presented case supports an expectant attitude given the possibility of a spontaneous regression after the biopsy, especially in small lesions. However, long-term follow-up is essential given the risk of recurrence.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613221135987"},"PeriodicalIF":0.9,"publicationDate":"2022-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/28/56/10.1177_20363613221135987.PMC9583201.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40567276","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 : 2022-10-03eCollection Date: 2022-01-01DOI: 10.1177/20363613221130155
Sungchul Ko, Kye Hoon Park, Ji-Hye Lee, Ki Nam Park
Metastasis of pleomorphic adenoma (PA) is rare and usually presented as a locoregional recurrence developed many years after excision of the primary tumor although the PA is the most common neoplasm in the parotid gland. We described a case of a 48-year-old male with a parotid tumor with multiple enlarged ipsilateral lymph nodes which suggested a malignancy. The tumors had been neither evaluated nor excised and preoperative evaluation revealed benign PA in both lesions. After the complete surgical excision, the final pathology was notable for benign PA with metastasis to regional lymph nodes. At 1 year follow up he was clinically and radiographically free of disease. This implies that pleomorphic adenoma can occur as initially metastasis to regional lymph node even though benign neoplasm.
{"title":"A case of initially metastasizing pleomorphic adenoma of parotid gland.","authors":"Sungchul Ko, Kye Hoon Park, Ji-Hye Lee, Ki Nam Park","doi":"10.1177/20363613221130155","DOIUrl":"https://doi.org/10.1177/20363613221130155","url":null,"abstract":"<p><p>Metastasis of pleomorphic adenoma (PA) is rare and usually presented as a locoregional recurrence developed many years after excision of the primary tumor although the PA is the most common neoplasm in the parotid gland. We described a case of a 48-year-old male with a parotid tumor with multiple enlarged ipsilateral lymph nodes which suggested a malignancy. The tumors had been neither evaluated nor excised and preoperative evaluation revealed benign PA in both lesions. After the complete surgical excision, the final pathology was notable for benign PA with metastasis to regional lymph nodes. At 1 year follow up he was clinically and radiographically free of disease. This implies that pleomorphic adenoma can occur as initially metastasis to regional lymph node even though benign neoplasm.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613221130155"},"PeriodicalIF":0.9,"publicationDate":"2022-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/50/bd/10.1177_20363613221130155.PMC9536096.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33496269","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}
Inflammatory myofibroblastic tumor (IMT) is an uncommon chest pathology. Treatment primarily focuses on surgical resection for diagnostic and therapeutic purposes. However, there are instances in which alternative therapies with steroids, chemotherapy, or radiation are necessary. We discuss a case of recurrent IMT for which very low dose radiation proved an effective treatment.
{"title":"Very low-dose radiation therapy for management of inflammatory myofibroblastic tumor of the lung.","authors":"Nimeh Najjar, Hamel Patel, Scott Steinberg, Brett Baskovich, Stephanie Rothweiler, Bradford Hoppe","doi":"10.1177/20363613221106270","DOIUrl":"https://doi.org/10.1177/20363613221106270","url":null,"abstract":"<p><p>Inflammatory myofibroblastic tumor (IMT) is an uncommon chest pathology. Treatment primarily focuses on surgical resection for diagnostic and therapeutic purposes. However, there are instances in which alternative therapies with steroids, chemotherapy, or radiation are necessary. We discuss a case of recurrent IMT for which very low dose radiation proved an effective treatment.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613221106270"},"PeriodicalIF":0.9,"publicationDate":"2022-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/24/a4/10.1177_20363613221106270.PMC9290091.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40610937","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 : 2022-07-08eCollection Date: 2022-01-01DOI: 10.1177/20363613221112432
Joshua A Cuoco, Andrew C Strohman, Brittany M Stopa, Michael S Stump, John J Entwistle, Mark R Witcher, Adeolu L Olasunkanmi
Cortical ependymomas are currently not considered a subgroup of supratentorial ependymomas; however, there is a growing body of literature investigating the natural history of these lesions compared to supratentorial ependymomas. We performed a systematic literature review of cortical ependymomas with a focus on the natural history, clinical characteristics, and clinical outcomes of these lesions as compared to supratentorial ependymomas. Our search revealed 153 unique cases of cortical ependymomas. The mean age on presentation was 21.2 years. Males and females comprised 58.8% (90/153) and 41.2% (63/153) of cases, respectively. The most common presenting symptom was seizure activity occurring in 44.4% of the cohort (68/153). The recently recognized C11orf95-RELA fusion was identified in 13.7% of the cohort (21/153) and 95.5% of cases (21/22) reporting molecular characterization. World Health Organization grades 2 and 3 were reported in 52.3% (79/151) and 47.7% (72/151) of cases, respectively. The frontal lobe was involved in the majority of cases (54.9%, 84/153). Gross total resection was achieved in 80.4% of cases (123/153). Tumor recurrence was identified in 27.7% of cases (39/141). Mean clinical follow-up was 41.3 months. Mean overall survival of patients who expired was 27.4 months whereas mean progression-free survival was 15.0 months. Comparatively, cortical ependymomas with C11orf95-RELA fusions and supratentorial ependymomas with C11orf95 RELA fusions exhibited differing clinical outcomes. Further studies with larger sample sizes are necessary to investigate the significance of RELA fusions on survival in cortical ependymomas and to determine whether cortical ependymomas with C11orf95-RELA fusions should be classified as a distinct entity.
{"title":"Supratentorial cortical ependymoma: A systematic literature review and case illustration.","authors":"Joshua A Cuoco, Andrew C Strohman, Brittany M Stopa, Michael S Stump, John J Entwistle, Mark R Witcher, Adeolu L Olasunkanmi","doi":"10.1177/20363613221112432","DOIUrl":"https://doi.org/10.1177/20363613221112432","url":null,"abstract":"<p><p>Cortical ependymomas are currently not considered a subgroup of supratentorial ependymomas; however, there is a growing body of literature investigating the natural history of these lesions compared to supratentorial ependymomas. We performed a systematic literature review of cortical ependymomas with a focus on the natural history, clinical characteristics, and clinical outcomes of these lesions as compared to supratentorial ependymomas. Our search revealed 153 unique cases of cortical ependymomas. The mean age on presentation was 21.2 years. Males and females comprised 58.8% (90/153) and 41.2% (63/153) of cases, respectively. The most common presenting symptom was seizure activity occurring in 44.4% of the cohort (68/153). The recently recognized <i>C11orf95-RELA</i> fusion was identified in 13.7% of the cohort (21/153) and 95.5% of cases (21/22) reporting molecular characterization. World Health Organization grades 2 and 3 were reported in 52.3% (79/151) and 47.7% (72/151) of cases, respectively. The frontal lobe was involved in the majority of cases (54.9%, 84/153). Gross total resection was achieved in 80.4% of cases (123/153). Tumor recurrence was identified in 27.7% of cases (39/141). Mean clinical follow-up was 41.3 months. Mean overall survival of patients who expired was 27.4 months whereas mean progression-free survival was 15.0 months. Comparatively, cortical ependymomas with <i>C11orf95</i>-<i>RELA</i> fusions and supratentorial ependymomas with <i>C11orf95 RELA</i> fusions exhibited differing clinical outcomes. Further studies with larger sample sizes are necessary to investigate the significance of <i>RELA</i> fusions on survival in cortical ependymomas and to determine whether cortical ependymomas with <i>C11orf95</i>-<i>RELA</i> fusions should be classified as a distinct entity.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613221112432"},"PeriodicalIF":0.9,"publicationDate":"2022-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/bd/87/10.1177_20363613221112432.PMC9274435.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40506725","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 : 2022-07-04eCollection Date: 2022-01-01DOI: 10.1177/20363613221110836
Robert M Tungate, Kristi Lara, Dakshesh Patel, Alexander Fedenko, James Hu
Ewing sarcoma is a primitive neuroectodermal tumor which seldom presents with primary disease in people over age 40 and outside of the appendicular or axial skeleton. We examine a case of primary thoracic Ewing Sarcoma diagnosed initially by CT-guided biopsy in a woman at the age of 74 years. The disease progressed after initial combined modality therapy consisting of neoadjuvant chemotherapy, surgical resection, and adjuvant radiation therapy and two additional courses of multiagent chemotherapy. After relapse of her disease, subsequent second- and third-line systemic agents which included chemotherapy and targeted agents were given with disease stabilization achieved now over 30 months from initial diagnosis. To our knowledge, this is the first report of a primary pulmonary Ewing sarcoma diagnosed in a patient greater than 70 years of age in whom multiple remissions have been achieved with tolerable toxicities.
{"title":"Remission of a Primary, Recurrent Thoracic Ewing Sarcoma in a 74-year-old woman.","authors":"Robert M Tungate, Kristi Lara, Dakshesh Patel, Alexander Fedenko, James Hu","doi":"10.1177/20363613221110836","DOIUrl":"10.1177/20363613221110836","url":null,"abstract":"<p><p>Ewing sarcoma is a primitive neuroectodermal tumor which seldom presents with primary disease in people over age 40 and outside of the appendicular or axial skeleton. We examine a case of primary thoracic Ewing Sarcoma diagnosed initially by CT-guided biopsy in a woman at the age of 74 years. The disease progressed after initial combined modality therapy consisting of neoadjuvant chemotherapy, surgical resection, and adjuvant radiation therapy and two additional courses of multiagent chemotherapy. After relapse of her disease, subsequent second- and third-line systemic agents which included chemotherapy and targeted agents were given with disease stabilization achieved now over 30 months from initial diagnosis. To our knowledge, this is the first report of a primary pulmonary Ewing sarcoma diagnosed in a patient greater than 70 years of age in whom multiple remissions have been achieved with tolerable toxicities.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613221110836"},"PeriodicalIF":0.9,"publicationDate":"2022-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/bc/de/10.1177_20363613221110836.PMC9260570.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40579243","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 : 2022-06-24eCollection Date: 2022-01-01DOI: 10.1177/20363613221110837
Salsabil Nasri, Mohamed Hedi Mraidha, Mehdi Ben Abdelkrim, Sabri Youssef, Fehmi Hamila, Mohamed Amine Elghali
Background: Schwannomas are mesenchymal tumors arising from neural sheath cells and whose diagnosis is based on immunohistochemistery. The digestive and especially colonic location of this tumor is rare. Commonly described in elderly patient, their malignancy is unusual.
Case report: We report the case of a 23-year-old girl, with learning disability, operated in emergency for acute peritonitis. Peroperatively, we discovered a peritonitis secondary to a bulky perforated cecal tumor. We performed a right colectomy and an ileostomy. The posterior margin of the resection was macroscopically involved. The diagnosis of colonic schwannoma was confirmed with immunopathological examination of the surgical specimen. Surgical exploration 4 months later and morphological investigations during 2 years showed stability of the tumoral residue in the right iliac fossa. However, we noticed, on CT scanning control 2 years postoperatively, the appearance of a mesenteric recurrence. Exploratory laparotomy confirmed the unresectability of this mesenteric mass and showed the presence of multiple parietal nodules whose biopsies revealed their neurofibromatous nature. The 4 year follow-up of the patient didn't reveal any complication rather than need to right nephrostomy due to ureter compression by the primary tumoral residue.
Discussion: Colonic schwannoma is a rare disease, commonly described in uncomplicated stages. The learning disability of our patient had favorized the absence of declared symptoms and promoted the evolution of the cecal swhannoma until its perforation. This complication was not described before. Local recurrence has been also rarely reported in the literature. Association with neurofibroma may be hereditary in a context of neurofibromatosis or sporadic.
Conclusion: Colonic schwannoma may have polymorphic presentation mimicking malignant tumor in such cases. Not treated in time, it can lead to severe complications, such as tumoral perforation. Surgical resection remains the mainstay treatment. The slow evolutionary genius of schwannoma, even when incompletely resected by necessity, confirms its good prognosis.
{"title":"Colonic schwannoma: A case of unusual presentation and outcome.","authors":"Salsabil Nasri, Mohamed Hedi Mraidha, Mehdi Ben Abdelkrim, Sabri Youssef, Fehmi Hamila, Mohamed Amine Elghali","doi":"10.1177/20363613221110837","DOIUrl":"https://doi.org/10.1177/20363613221110837","url":null,"abstract":"<p><strong>Background: </strong>Schwannomas are mesenchymal tumors arising from neural sheath cells and whose diagnosis is based on immunohistochemistery. The digestive and especially colonic location of this tumor is rare. Commonly described in elderly patient, their malignancy is unusual.</p><p><strong>Case report: </strong>We report the case of a 23-year-old girl, with learning disability, operated in emergency for acute peritonitis. Peroperatively, we discovered a peritonitis secondary to a bulky perforated cecal tumor. We performed a right colectomy and an ileostomy. The posterior margin of the resection was macroscopically involved. The diagnosis of colonic schwannoma was confirmed with immunopathological examination of the surgical specimen. Surgical exploration 4 months later and morphological investigations during 2 years showed stability of the tumoral residue in the right iliac fossa. However, we noticed, on CT scanning control 2 years postoperatively, the appearance of a mesenteric recurrence. Exploratory laparotomy confirmed the unresectability of this mesenteric mass and showed the presence of multiple parietal nodules whose biopsies revealed their neurofibromatous nature. The 4 year follow-up of the patient didn't reveal any complication rather than need to right nephrostomy due to ureter compression by the primary tumoral residue.</p><p><strong>Discussion: </strong>Colonic schwannoma is a rare disease, commonly described in uncomplicated stages. The learning disability of our patient had favorized the absence of declared symptoms and promoted the evolution of the cecal swhannoma until its perforation. This complication was not described before. Local recurrence has been also rarely reported in the literature. Association with neurofibroma may be hereditary in a context of neurofibromatosis or sporadic.</p><p><strong>Conclusion: </strong>Colonic schwannoma may have polymorphic presentation mimicking malignant tumor in such cases. Not treated in time, it can lead to severe complications, such as tumoral perforation. Surgical resection remains the mainstay treatment. The slow evolutionary genius of schwannoma, even when incompletely resected by necessity, confirms its good prognosis.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613221110837"},"PeriodicalIF":0.9,"publicationDate":"2022-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/7a/8c/10.1177_20363613221110837.PMC9237920.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40468230","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 : 2022-01-01DOI: 10.1177/20363613221079754
Michael P. Fice, Linus Lee, Pavan Kottamasu, Abdullah Almajnooni, Matthew R. Cohn, Charles A. Gusho, S. Gitelis, Alan T. Blank
Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant soft tissue sarcoma (STS) that accounts for less than 3% of all soft tissue tumors. The conventional treatment for primary EMC is wide local excision with or without radiation therapy. Materials and Methods This study was a retrospective review of all EMC cases treated within a single institution between 1992 and 2019. EMC was diagnosed using a combination of histologic morphology and immunostaining, with confirmatory fluorescent in situ hybridization. Overall survival (OS) and disease-specific survival (DSS) were defined using Kaplan–Meier analysis. Results Fifteen patients were evaluated, including 11 males and four females. The average age at presentation was 51.7 ± 20.4 years and the mean follow-up time was 61.5 months (range, 5–286 months). The average resected tumor size at largest dimension was 7.14 cm (range, 2.4–18.7). Twelve of fifteen (80%) patients underwent wide local excision, and nine of the twelve (75%) underwent local radiation therapy. The 1-, 5-, and 10-year OS was 80% (95% CI, 59.8–100), 72% (95% CI, 48.5–95.5), and 72% (95% CI, 48.5–95.5), respectively. The 1-, 5-, and 10-year DSS was 92.3% (95% CI, 77.8–100), 83.1% (95% CI, 61.5–100), and 83.1% (95% CI, 61.5–100), respectively. At last follow-up, 11 patients were alive and ten (90.9%) were disease free. Conclusions Extraskeletal myxoid chondrosarcoma is a very rare STS most often seen in males and in the extremities. Our cohort was too small to provide meaningful statistical analysis; however, we observed lower rates of local recurrence in patients treated with radiation.
{"title":"Extraskeletal myxoid chondrosarcoma: A case series and review of the literature","authors":"Michael P. Fice, Linus Lee, Pavan Kottamasu, Abdullah Almajnooni, Matthew R. Cohn, Charles A. Gusho, S. Gitelis, Alan T. Blank","doi":"10.1177/20363613221079754","DOIUrl":"https://doi.org/10.1177/20363613221079754","url":null,"abstract":"Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant soft tissue sarcoma (STS) that accounts for less than 3% of all soft tissue tumors. The conventional treatment for primary EMC is wide local excision with or without radiation therapy. Materials and Methods This study was a retrospective review of all EMC cases treated within a single institution between 1992 and 2019. EMC was diagnosed using a combination of histologic morphology and immunostaining, with confirmatory fluorescent in situ hybridization. Overall survival (OS) and disease-specific survival (DSS) were defined using Kaplan–Meier analysis. Results Fifteen patients were evaluated, including 11 males and four females. The average age at presentation was 51.7 ± 20.4 years and the mean follow-up time was 61.5 months (range, 5–286 months). The average resected tumor size at largest dimension was 7.14 cm (range, 2.4–18.7). Twelve of fifteen (80%) patients underwent wide local excision, and nine of the twelve (75%) underwent local radiation therapy. The 1-, 5-, and 10-year OS was 80% (95% CI, 59.8–100), 72% (95% CI, 48.5–95.5), and 72% (95% CI, 48.5–95.5), respectively. The 1-, 5-, and 10-year DSS was 92.3% (95% CI, 77.8–100), 83.1% (95% CI, 61.5–100), and 83.1% (95% CI, 61.5–100), respectively. At last follow-up, 11 patients were alive and ten (90.9%) were disease free. Conclusions Extraskeletal myxoid chondrosarcoma is a very rare STS most often seen in males and in the extremities. Our cohort was too small to provide meaningful statistical analysis; however, we observed lower rates of local recurrence in patients treated with radiation.","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43245665","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}
Pub Date : 2022-01-01DOI: 10.1177/20363613221103751
Mehdi Salehipour, Mohammad Hossein Anbardar, Bita Geramizadeh, Hamed Jafari, Ali Zare, Ali Adib
Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare tumor of the soft tissue, usually located in lower extremities. There are rarely tumors reported in other anatomic locations. Herein, we report retroperitoneal PHAT in a male patient. A 41-year-old man was referred to our clinic due to an incidentally found retroperitoneal mass. Computed tomography (CT) scan showed a solid hypoechoic lesion containing fat component and calcified elements measuring about 80*72*45 mm in the right lower quadrant (RLQ) of the abdomen. Magnetic resonance imaging (MRI) showed circumscribe lesion measuring about 60 x 48 mm with partial enhancement and fat component. In pelvic exploration, a large mass was found that had encased the right external iliac artery and vein. Therefore, the mass and its surrounding iliac vessels were excised and removed en block. Then, the external iliac vessels were reconstructed with Gortex graft. No recurrence was found in 1 month and 3 months post-operation follow up. We report a pelvic retroperitoneal PHAT as a rare location of this tumor. It seems that PHAT must be considered in differential diagnosis in patients with soft tissue tumors in the pelvic cavity.
摘要多形性透明化血管扩张瘤(PHAT)是一种罕见的软组织肿瘤,通常位于下肢。在其他解剖部位很少有肿瘤的报道。在此,我们报告一位男性患者腹膜后PHAT。一位41岁的男性因偶然发现腹膜后肿块而被转介到我们诊所。CT示腹部右下象限(RLQ)一实性低回声病灶,含脂肪成分和钙化元素,大小约80*72*45 mm。磁共振成像(MRI)显示病灶范围约60 x 48 mm,局部增强,脂肪成分。在盆腔探查中,发现一个大肿块包裹了右侧髂外动脉和静脉。因此,肿块及其周围的髂血管被切除并整体切除。然后用Gortex移植物重建髂外血管。术后1个月及3个月随访均无复发。我们报告盆腔腹膜后PHAT是一个罕见的肿瘤位置。因此,在盆腔软组织肿瘤患者的鉴别诊断中必须考虑到PHAT。
{"title":"Pelvic retroperitoneal pleomorphic hyalinizing angiectatic tumor: A case report and review of literature.","authors":"Mehdi Salehipour, Mohammad Hossein Anbardar, Bita Geramizadeh, Hamed Jafari, Ali Zare, Ali Adib","doi":"10.1177/20363613221103751","DOIUrl":"https://doi.org/10.1177/20363613221103751","url":null,"abstract":"<p><p>Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare tumor of the soft tissue, usually located in lower extremities. There are rarely tumors reported in other anatomic locations. Herein, we report retroperitoneal PHAT in a male patient. A 41-year-old man was referred to our clinic due to an incidentally found retroperitoneal mass. Computed tomography (CT) scan showed a solid hypoechoic lesion containing fat component and calcified elements measuring about 80*72*45 mm in the right lower quadrant (RLQ) of the abdomen. Magnetic resonance imaging (MRI) showed circumscribe lesion measuring about 60 x 48 mm with partial enhancement and fat component. In pelvic exploration, a large mass was found that had encased the right external iliac artery and vein. Therefore, the mass and its surrounding iliac vessels were excised and removed en block. Then, the external iliac vessels were reconstructed with Gortex graft. No recurrence was found in 1 month and 3 months post-operation follow up. We report a pelvic retroperitoneal PHAT as a rare location of this tumor. It seems that PHAT must be considered in differential diagnosis in patients with soft tissue tumors in the pelvic cavity.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"14 ","pages":"20363613221103751"},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/51/60/10.1177_20363613221103751.PMC9150221.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10252651","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 : 2022-01-01DOI: 10.1177/20363613221080549
D. Massaras, E. Kontis, K. Stamatis, E. Zampeli, D. Myoteri, Elias Primetis, E. Pantiora, G. Fragulidis
Leiomyosarcomas (LMS) are mesenchymal tumors of smooth muscle origin. Approximately 20% of leiomyosarcomas are found in the GI tract, and account for 1–2% of GI malignancies. Within the gastrointestinal tract, the small intestine is the most common site of presentation followed by the colon. They are often diagnosed incidentally during abdominal pain investigation, and they usually present in the fifth decade of life. In the past, the diagnostic differentiation between leiomyosarcomas of gastrointestinal tract and gastrointestinal stromal tumors (GISTs) was very challenging as they share common microscopic appearance. Nowadays, these tumors are diagnosed by immunohistochemical methods as they are positive for smooth muscle markers actin (SMA) and desmin, negative for GIST markers such as receptor tyrosine kinase (KIT), CD34, DOG1, and negative for the schwannoma marker S100 protein. In the current literature, most cases of intestinal leiomyosarcomas are localized in the small intestine, and there are no reports of synchronous liver metastases at the time of presentation of the primary tumor. Herein, we present a patient who was admitted in our department, with the diagnosis of primary leiomyosarcoma of the colon and synchronous liver metastasis.
{"title":"Primary leiomyosarcoma of the colon with synchronous liver metastasis","authors":"D. Massaras, E. Kontis, K. Stamatis, E. Zampeli, D. Myoteri, Elias Primetis, E. Pantiora, G. Fragulidis","doi":"10.1177/20363613221080549","DOIUrl":"https://doi.org/10.1177/20363613221080549","url":null,"abstract":"Leiomyosarcomas (LMS) are mesenchymal tumors of smooth muscle origin. Approximately 20% of leiomyosarcomas are found in the GI tract, and account for 1–2% of GI malignancies. Within the gastrointestinal tract, the small intestine is the most common site of presentation followed by the colon. They are often diagnosed incidentally during abdominal pain investigation, and they usually present in the fifth decade of life. In the past, the diagnostic differentiation between leiomyosarcomas of gastrointestinal tract and gastrointestinal stromal tumors (GISTs) was very challenging as they share common microscopic appearance. Nowadays, these tumors are diagnosed by immunohistochemical methods as they are positive for smooth muscle markers actin (SMA) and desmin, negative for GIST markers such as receptor tyrosine kinase (KIT), CD34, DOG1, and negative for the schwannoma marker S100 protein. In the current literature, most cases of intestinal leiomyosarcomas are localized in the small intestine, and there are no reports of synchronous liver metastases at the time of presentation of the primary tumor. Herein, we present a patient who was admitted in our department, with the diagnosis of primary leiomyosarcoma of the colon and synchronous liver metastasis.","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48957682","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}
Pub Date : 2022-01-01DOI: 10.1177/20363613221147470
Nikolaos Tasis, Antonia A Prountzopoulou, Evangelia Skafida, Maria Arnaouti, Theodoros Tsirlis, Aris Plastiras, Dimitrios K Manatakis, Niki Arnogiannaki, Dimitrios P Korkolis
Pancreatic mature cystic teratomas are very rare with limited cases found in the literature. These lesions raise a diagnostic challenge and complicate the surgical approach not only because of their anatomic position but also because of their ever-growing size. An elusive diagnosis, usually leads to the operative theatre where surgical resection takes place. We present a rare case of a large pancreatic cystic teratoma extending into the mediastinum in a 29-year-old woman which was succesfully managed with en-bloc distal pancreatectomy and spleenectomy.
{"title":"Giant teratoma of the pancreas expanding to the mediastinum: Rare tumor and literature review.","authors":"Nikolaos Tasis, Antonia A Prountzopoulou, Evangelia Skafida, Maria Arnaouti, Theodoros Tsirlis, Aris Plastiras, Dimitrios K Manatakis, Niki Arnogiannaki, Dimitrios P Korkolis","doi":"10.1177/20363613221147470","DOIUrl":"https://doi.org/10.1177/20363613221147470","url":null,"abstract":"<p><p>Pancreatic mature cystic teratomas are very rare with limited cases found in the literature. These lesions raise a diagnostic challenge and complicate the surgical approach not only because of their anatomic position but also because of their ever-growing size. An elusive diagnosis, usually leads to the operative theatre where surgical resection takes place. We present a rare case of a large pancreatic cystic teratoma extending into the mediastinum in a 29-year-old woman which was succesfully managed with en-bloc distal pancreatectomy and spleenectomy.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"14 ","pages":"20363613221147470"},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3b/bb/10.1177_20363613221147470.PMC9806373.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10494337","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}