Rare TumorsPub Date : 2024-07-24eCollection Date: 2024-01-01DOI: 10.1177/20363613241266047
Youssef Achour, Youness Bakali, Mahdi Bahij, Hamza Sekkat, Mouna Mhamdi Alaoui, Farid Sabbah, Abdelmalek Hrora, Mohammed Raiss
{"title":"Surgical management of giant retroperitoneal liposarcoma: Case report.","authors":"Youssef Achour, Youness Bakali, Mahdi Bahij, Hamza Sekkat, Mouna Mhamdi Alaoui, Farid Sabbah, Abdelmalek Hrora, Mohammed Raiss","doi":"10.1177/20363613241266047","DOIUrl":"10.1177/20363613241266047","url":null,"abstract":"<p><p>Tumors originating from soft tissues are uncommon, among these tumors, liposarcomas are the most frequent. These tumors remain asymptomatic for a long time, and only revealing themselves when they reach an important size. In such cases, treatment is difficult, requiring extensive surgery procedures that can excise several adjacent structures, potentially completed by adjuvant radiotherapy. Despite successful treatment, the recurrence rate remains very high. We report the case of a giant liposarcoma requiring a monobloc extensive resection involving the removal of the tumor, left kidney, left adrenal gland, and a portion of the posterior abdominal wall.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241266047"},"PeriodicalIF":0.9,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11271108/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141761604","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}
Rare TumorsPub Date : 2024-05-16eCollection Date: 2024-01-01DOI: 10.1177/20363613241255668
Joshua R Finlay, Gayathri Vijayakumar, Ankica Braun, Abdullah Almajnooni, Austin Yu, Ira Miller, George Kokosis, Dian Wang, Alan Blank, Steven Gitelis
{"title":"Enormous myxoid/round cell liposarcoma: A case report.","authors":"Joshua R Finlay, Gayathri Vijayakumar, Ankica Braun, Abdullah Almajnooni, Austin Yu, Ira Miller, George Kokosis, Dian Wang, Alan Blank, Steven Gitelis","doi":"10.1177/20363613241255668","DOIUrl":"10.1177/20363613241255668","url":null,"abstract":"<p><p>Myxoid/round cell liposarcomas (MRCLPS) are a rare soft tissue sarcoma. We report the largest sarcoma in our institutional history. We discuss the patient's surgical management and treatment of the tumor and challenges given its dimensions. Several complications arose following primary resection that were managed by a multidisciplinary team. Although MRCLPS can vary in size, large MRCLPS must be treated cautiously given the potential for complications. Additionally, multidisciplinary treatment of MRCLPS is essential in diagnosing and treating these complex cases.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241255668"},"PeriodicalIF":0.9,"publicationDate":"2024-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11100375/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141065472","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}
Rare TumorsPub Date : 2024-05-15eCollection Date: 2024-01-01DOI: 10.1177/20363613241255669
Daniel Nguyen, Nyein Nyein Htun, Cary Johnson
{"title":"Gigantic laryngeal schwannoma: A case report with literature review.","authors":"Daniel Nguyen, Nyein Nyein Htun, Cary Johnson","doi":"10.1177/20363613241255669","DOIUrl":"https://doi.org/10.1177/20363613241255669","url":null,"abstract":"<p><p>Laryngeal schwannoma is a rare benign nerve sheath tumor that is slow growing. The diagnosis is made from a combination of clinical, radiological, and histopathological findings, and the main method of treatment is resection. We report a case of a 69-year-old presenting with a neck mass causing stridor, dysphagia, and orthopnea. CT of the neck showed an enhancing mass measuring 6.3 cm and extending superior to the larynx. Emergent tracheostomy and mass resection were performed, and histopathology and immunohistochemical findings were obtained from the specimen supporting schwannoma. In conclusion, while rare, schwannoma should always be considered as a differential diagnosis for a laryngeal mass. More studies are needed to assess the size and prognosis of the tumor.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241255669"},"PeriodicalIF":0.9,"publicationDate":"2024-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11097735/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140959850","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}
{"title":"Respiratory epithelial adenomatoid hamartoma: A rare differential diagnosis of sinonasal mass.","authors":"Safa Nefzaoui, Imen Zoghlami, Jihene Gharsalli, Emna Sabehi, Nadia Romdhane, Imen Helal, Dorra Chiboub, Ines Hariga, Chiraz Mbarek","doi":"10.1177/20363613241255567","DOIUrl":"https://doi.org/10.1177/20363613241255567","url":null,"abstract":"<p><p>Sino-nasal respiratory epithelial adenomatoid hamartomas (REAHs) are rare entity. They are benign tumors with excellent results after complete excision. We report a case of a 57-year-old male with a history of endoscopic surgery for right nasal polyps 20 years ago. The patient presented nasal obstruction that persisted for 10 years without anosmia nor epistaxis. Nasal endoscopy found a tissular mass filling the right nasal cavity extending to the nasopharynx. CT scan and MRI demonstrated soft tissue opacification of the right maxillary sinus and the homolateral anterior ethmoid cells with extension to the nasal cavity. The suspected diagnosis on imaging was an Inverted papilloma with a wide implantation base on the posterior part of the nasal septum. No endocranial or orbital extension was noted. The patient underwent endoscopic sinus surgery with complete extirpation of the tumor and a right ethmoidectomy. Histopathological assessment showed features consistent with REAH. No recurrence was noted at 1 year follow-up.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241255567"},"PeriodicalIF":0.9,"publicationDate":"2024-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11097725/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140959854","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}
{"title":"Outcomes of pediatric extracranial germ cell tumors: A single center experience in a developing country.","authors":"Symbat Saliyeva, Riza Boranbayeva, Minira Bulegenova, Bakhram Zhumadullayev, Gaukhar Nurzhanova, Lyazat Manzhuova","doi":"10.1177/20363613231216567","DOIUrl":"https://doi.org/10.1177/20363613231216567","url":null,"abstract":"<p><p>The purpose of this study was to analyze the outcomes of extracranial GCT in children in a developing country and to assess prognostic factors. The data on 141 children (<18 years old) with extracranial GCT, confirmed histopathologically, collected over the past 9 years (from February 2013 to June 2022) were retrospectively studied. The patients underwent the same therapy with platinum-containing chemotherapy regimens. In the malignant GCT group, OS and EFS were 81.0 ± 4% and 73 ± 5%, respectively. OS and EFS in the teratoma group were 90 ± 5% and 85 ± 6%. In univariate analysis, parameters like stage of disease, tumor localization, AFP level ≥10,000 ng/mL, serum AFP kinetics and resection status were found to be statistically significant prognostic factors. In the multivariate analysis, the significant adverse factors were the resection status, initial AFP level ≥10,000 ng/mL and serum AFP kinetics slow down (<i>p</i> = .000). Good survival rates can be achieved in developing countries with adequate compliance with treatment protocols. The analysis demonstrates high efficacy of platinum-containing chemotherapy regimens. In our opinion, the protocol used in high-income countries can be implemented in low-income countries with the financial support from the government. The qualification of specialists is also important.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613231216567"},"PeriodicalIF":0.9,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10986168/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140871739","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}
Rare TumorsPub Date : 2024-03-28eCollection Date: 2024-01-01DOI: 10.1177/20363613241242570
Hayfa Chahdoura, Alia Mousli, Ghaiet El Fidaa Noubigh, Safia Yahyaoui, Rim Abidi, Nasr Chiraz
{"title":"Ewing's sarcoma of the parotid gland: A rare entity with review of the literature.","authors":"Hayfa Chahdoura, Alia Mousli, Ghaiet El Fidaa Noubigh, Safia Yahyaoui, Rim Abidi, Nasr Chiraz","doi":"10.1177/20363613241242570","DOIUrl":"10.1177/20363613241242570","url":null,"abstract":"<p><strong>Background: </strong>Ewing's sarcoma is a rare malignant entity. Extraosseous Ewing's sarcoma (EES) of the head and neck region is a rare occurrence, and Ewing's sarcoma of the parotid gland is even rarer. To the best of our knowledge, we reported the first case of extraskeletal ES originating from the parotid gland in the Tunisian literature.</p><p><strong>Case report: </strong>We report a rare case of EES of the parotid gland in a 35-year-old female. She presented with left parotid tumefaction. Physical examination revealed solid and fixed mass associated with facial paralysis. Magnetic resonance imaging illustrated a left intra-parotid process occupying the entire gland measuring 42 mm infiltrating the masseter and pterygoid muscles. The patient had a total left parotidectomy with ipsilateral triangular lymph node dissection. The definitive pathological examination and the immunohistochemical staining confirmed a primary peripheral neuroectodermal tumor or PNET with the presence of a specific EWING/PNET-type translocation in 60% of the tumor cells. She had an adjuvant chemotherapy (four cycles of vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide and etoposide) followed by external radiotherapy.</p><p><strong>Conclusion: </strong>A clinical and radiological follow-up by cervical MRI was done every 3 months and The 10-month follow-up showed no locoregional and distant recurrence.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241242570"},"PeriodicalIF":0.9,"publicationDate":"2024-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10979530/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140337061","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}
Rare TumorsPub Date : 2024-03-27eCollection Date: 2024-01-01DOI: 10.1177/20363613241242705
Alia Mousli, Ferdaous Friaa, Emna Boudhina, Selma Kamoun, Rim Abidi, Chiraz Nasr
{"title":"Spindle cell carcinomaof the larynx: A case report and literature review.","authors":"Alia Mousli, Ferdaous Friaa, Emna Boudhina, Selma Kamoun, Rim Abidi, Chiraz Nasr","doi":"10.1177/20363613241242705","DOIUrl":"10.1177/20363613241242705","url":null,"abstract":"<p><strong>Introduction: </strong>Spindle cell carcinoma (SCC), also called sarcomatoid carcinoma or carcinosarcoma, represents a rare variant of squamous cell carcinoma that may involve multiple organs, including the larynx. It is a mixed tumor, composed of both malignant epithelial and mesenchymal elements. As the larynx appears to be an unusual site, we report a case of treated SCC laryngeal as well as a literature review of such tumor.</p><p><strong>Observation: </strong>A 72-year-old male, with 2 months' history of a progressive worsening dysphonia, was examined. The patient examination showed no cervical adenopathy, while direct laryngoscopy detected a voluminous budding formation of the anterior commissure. A biopsy was performed, in which an undifferentiated carcinoma was identified. The computed tomography (CT) confirmed the existence of a glottic tumor, which completely obstructs the lumen of the larynx. No metastasis were noticed elsewhere on the PET-CT. The tumor was then classified as T3 N0 M0. The patient underwent a total laryngectomy, thyroidectomy and neck dissection. The definitive histopathological analysis confirmed the diagnosis of SCC of the larynx with negative surgical margins. Afterward, the patient underwent adjuvant radiotherapy (RT) on the tumor bed.</p><p><strong>Conclusion: </strong>Primary laryngeal SCC is a very rare malignancy with a tendency to occur with controlateral lymph node metastasis at an early stage of the disease. Combining surgery and postoperative RT is recommended due to its aggressive profile.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241242705"},"PeriodicalIF":0.9,"publicationDate":"2024-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10976491/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140319505","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}
Rare TumorsPub Date : 2024-03-27eCollection Date: 2024-01-01DOI: 10.1177/20363613241242572
John Adi Ashindoitiang, Victor Ikechukwu Canice Nwagbara, Theophilus Ipeh Ugbem, Nkami Joseph Enya, Ikechimka Ogbugbanduchi Aniebo, Joseph Christopher Ekong, Sidney Esotu Obinna, Maurice Efana Asuquo
{"title":"Extra-gastrointestinal stromal tumour of the lesser omentum presenting as a massive intra-abdominal swelling: A case report and literature review.","authors":"John Adi Ashindoitiang, Victor Ikechukwu Canice Nwagbara, Theophilus Ipeh Ugbem, Nkami Joseph Enya, Ikechimka Ogbugbanduchi Aniebo, Joseph Christopher Ekong, Sidney Esotu Obinna, Maurice Efana Asuquo","doi":"10.1177/20363613241242572","DOIUrl":"10.1177/20363613241242572","url":null,"abstract":"<p><p>Extra gastrointestinal stromal tumour(EGIST) is rare and is regarded as gastrointestinal stromal tumour(GIST) that originates outside of the gastrointestinal tract. They originate from other intraabdominal tissues such as the omentum, mesentery and peritoneum. The cell of origin is the interstitial cell of Cajal(ICC), a pacemaker cell that controls gastrointestinal peristalsis and the tumor is characterized by the expression of KIT(CD117) a transmembrane tyrosine kinase receptor. Here, a 49-year-old female who presented with a 6 month history of abdominal pain, progressive abdominal swelling and the presence of an upper abdominal mass. She had surgical resection and adjuvant imatinib for the intraabdominal mass diagnosed on histology and immunohistochemistry as EGIST of the lesser omentum. Serial CT 14 months after surgery revealed no evidence of recurrence or metastasis. Clinicians should consider EGIST in the differential diagnosis of abdominal masses.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241242572"},"PeriodicalIF":0.9,"publicationDate":"2024-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10976504/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140319504","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}
Rare TumorsPub Date : 2024-03-23eCollection Date: 2024-01-01DOI: 10.1177/20363613241242397
Wangpan Shi, Timothy Law, Kevin Thomas Brumund, Jennifer Chang, Charmi Patel, Grace Lin, Jingjing Hu
{"title":"Low-grade mucoepidermoid carcinoma mimicking benign cystic lesions in the salivary gland: A diagnostic dilemma.","authors":"Wangpan Shi, Timothy Law, Kevin Thomas Brumund, Jennifer Chang, Charmi Patel, Grace Lin, Jingjing Hu","doi":"10.1177/20363613241242397","DOIUrl":"10.1177/20363613241242397","url":null,"abstract":"<p><p>Mucoepidermoid carcinoma (MEC) is a common malignancy arising in the parotid gland. The diagnosis of MEC is typically based on its morphological features alone, characteristically containing mucocytes, intermediate cells and epidermoid cells. However, when cystic degeneration is diffuse, it is challenging to distinguish MEC from other benign cystic tumors. This is a case report of a 58-year-old Caucasian man who presented with a parotid mass. H&E sections of the mass reveal multiloculated cysts lined by bland-looking epithelium with only rare papillary architectures. The papillary proliferation contains mucocytes, and epidermoid cells highlighted by the p63 immunohistochemistry study. The diagnosis was confirmed by FISH result of positive MAML2 (11q21) rearrangement. Patient underwent parotidectomy and is disease-free 6 months post-surgery. MEC with cystic degeneration is a common diagnostic pitfall which can mimic many benign lesions in the salivary gland. We present a rare case with MEC with extensive cystic change, its molecular and pathologic findings and review the diagnostic features of MEC, its benign mimickers and useful tools for distinguishing these entities.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241242397"},"PeriodicalIF":0.9,"publicationDate":"2024-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10960343/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140207873","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}
Rare TumorsPub Date : 2024-03-21eCollection Date: 2024-01-01DOI: 10.1177/20363613241242383
John Adi Ashindoitiang, Victor Ikechukwu Canice Nwagbara, Theophilus Ugbem Ipeh, Ben Chukwu, Henry Olaleye Obanife, Alfred Ottoh Eyong, Maurice Efana Asuquo
{"title":"Retroperitoneal mixed neuroendocrine non-neuroendocrine neoplasm: A case report and review of literature.","authors":"John Adi Ashindoitiang, Victor Ikechukwu Canice Nwagbara, Theophilus Ugbem Ipeh, Ben Chukwu, Henry Olaleye Obanife, Alfred Ottoh Eyong, Maurice Efana Asuquo","doi":"10.1177/20363613241242383","DOIUrl":"10.1177/20363613241242383","url":null,"abstract":"<p><p>The existence of both neuroendocrine and non-neuroendocrine histology in variable proportion in a lesion has been described by the World Health Organisation (WHO) as mixed neuroendocrine and non-neuroendocrine neoplasm (MiNEN). The pathogenesis of this tumour remains controversial but molecular studies point towards a common monoclonal origin. Tumours are classified as functioning and nonfunctioning based on substances secreted. The nonfunctioning tumours may be discovered due to its local effect. Presented is a 66-year-old male with an intra-abdominal mass, underwent laparotomy and excision biopsy with transient right lower limb lymphoedema. Histology confirmed retroperitoneal MiNEN with no evidence of tumour recurrence 12 months following surgery. MiNENs should be considered as a differential diagnosis in patients with intra-abdominal mass. Surgical resection is recommended as this may offer the best treatment option.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241242383"},"PeriodicalIF":0.9,"publicationDate":"2024-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10958797/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140207874","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}