scholarly journals Extraskeletal Mesenchymal Chondrosarcoma, a Rare Entity with Unusual Metastases: A Case Report

2021 ◽  
Vol 7 (2) ◽  
Author(s):  
Afaque Ali ◽  
Sara Rehman ◽  
Kashif Siddique

Introduction: Extraskeletal mesenchymal chondrosarcoma (ESMC) is rare, aggressive, and high grade malignant tumors originating from soft tissues. It carries a poor prognosis with a tendency for local recurrence and distant metastasis, necessitating long-term follow-up. The most common sites for metastasis are the lungs, bones, and lymph nodes. Meanwhile, pancreatic metastases are extremely rare. Case Description: A 35-year-old female presented with a history of wide local excision for the left upper limb mass; histopathology showed ESMC. She was on surveillance with a computed tomography scan of the thorax and magnetic resonance imaging of the left upper limb at 3-months intervals until she developed vertebral and pancreatic lesions after 6 months post-surgery. No pulmonary metastases were noted. Considering the unusual site for metastasis and to exclude the possibility of any second malignancy, bone biopsy, and endoscopic ultrasound-guided fine-needle aspiration was performed that confirmed metastases. Later she developed osseous metastases in the pelvis and femora. Practical Implication: Pancreatic metastasis from ESMC is extremely rare. In case of new visceral or osseous lesions in a patient with a past medical history of ESMC, the possibility of metastatic disease should be considered. A biopsy can be performed to confirm the diagnosis.

2021 ◽  
Vol 49 (5) ◽  
pp. 030006052110196
Author(s):  
Xiaotong Peng ◽  
Zhi Duan ◽  
Hongling Yin ◽  
Furong Dai ◽  
Huining Liu

Epithelioid angiosarcoma is a rare and highly aggressive soft tissue angiosarcoma most commonly arising in the deep soft tissues. Given that abundant vascular cavities anastomose with each other, most angiosarcomas prone to metastasis recur quickly, and the overall prognosis is poor. We report a 25-year-old woman at 24 weeks’ gestation who presented with a 1-month history of abdominal distension. Ultrasonography suggested a mass in the right adnexa, and she underwent two operations owing to uncontrolled intraperitoneal bleeding with progressive anemia. The right ovarian tumor and right adnexa were removed successively. Biopsy yielded a diagnosis of primary epithelioid angiosarcoma with mature cystic teratoma. The patient died from uncontrolled progressive bleeding 1 week after the second operation. This case revealed that epithelial angiosarcoma is a highly malignant endothelial cell tumor. The results of surgery and chemoradiotherapy tend to be poor, and the recurrence rate is high. The purpose of this study is to raise clinical awareness of epithelial angiosarcoma and its adverse events and to provide new ideas for the treatment of these adverse events. Immunohistochemical staining of pathological specimens can facilitate diagnosis. Pregnancy with malignant tumors may lead to rapid disease progression, extensive lesions, and a poor prognosis.


2018 ◽  
Vol 142 (11) ◽  
pp. 1421-1424 ◽  
Author(s):  
Komal Arora ◽  
Nicole D. Riddle

Extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma arising from soft tissues, mainly of the lower extremities, meninges, and orbits. It usually presents during the second to third decades of life, and has a slight predominance in females. Histologically, it has a typical biphasic pattern comprising small cells and islands of hyaline cartilage. It can pose a diagnostic challenge in small biopsy specimens where 1 of the 2 components can be absent. The prognosis is extremely variable; survival varies depending on the location of the tumor.


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Yuki Yamada ◽  
Kazuhiro Nishioka ◽  
Hirotaka Kajihara ◽  
Taketoshi Noguchi ◽  
Katsuhiko Naruse ◽  
...  

Phosphoglyceride crystal deposition disease (PGDD) is a rare disease entity that is characterized by phosphoglyceride crystal deposition that stimulates the formation of masses in soft tissue scars or bones. We report a case of PGDD in the pelvic soft tissues that recurred after initial surgical treatment. A 50-year-old woman was referred to our hospital for the evaluation of pelvic masses that were observed on an abdominal ultrasound. Magnetic resonance imaging (MRI) revealed masses in the pelvic region, with the largest being 10 cm in diameter. The masses were diagnosed as ovarian malignant tumors, and an exploratory laparotomy was performed. Operative findings revealed them to be foreign body granulomas, and the patient was diagnosed with PGDD. The patient had a history of cesarean delivery at the age of 24 years. PGDD is extremely rare, but it should be considered in the differential diagnosis of abdominal masses in patients with a history of abdominal surgery.


2022 ◽  
Vol 15 (1) ◽  
pp. e246375
Author(s):  
Himanshu Pruthi ◽  
Harish Bhujade ◽  
Reetu Kundu ◽  
Srinivasa GY

Mesenchymal chondrosarcoma (MC) is a rare cartilaginous tumour that occurs in the extraskeletal locations in about one-third of cases. It is aggressive in behaviour and may involve the lower extremities, central nervous system or spine. Mesenchymal tumours are known for distant metastasis; however, metastasis to bilateral kidneys after treatment has not been reported earlier. We present a case of a soft-tissue intramuscular MC of the thigh in a 38-year-old patient which had been surgically excised after neoadjuvant chemotherapy. The patient presented with bilateral dense calcified renal masses after 6 years, which were cytologically proven as MC metastases. In the evaluation of bilateral calcified renal masses in patients with a history of MC, metastasis should be considered.


2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S25-S25
Author(s):  
J Muldoon ◽  
L Warmke

Abstract Introduction/Objective Mesenchymal chondrosarcoma is a high-grade primitive mesenchymal tumor, which accounts for <5% of all chondrosarcomas and commonly affects young adults (peak incidence in second to third decade of life). The tumor has a widespread anatomic distribution, frequently involving the craniofacial bones as well as extraskeletal sites. The clinical presentation of an inguinal mass mimicking a thrombosed venous aneurysm is unusual and represents a potential diagnostic pitfall. Methods/Case Report A 42-year-old female with hypertension and obesity initially presented with a two-week history of left lower leg swelling. Venous doppler revealed presumed venous thrombosis, and she was prescribed apixaban while no history of coagulopathy, immobility, or recent surgery was noted. Two months later, she had residual swelling. Follow-up CT scan favored a large, peripherally thrombosed venous aneurysm arising from the left common femoral vein, while MRI showed a lobulated, inguinal soft tissue mass abutting the vein. Biopsy of the mass demonstrated a spindle cell mesenchymal neoplasm; subsequent resection revealed a pink-to-tan, well-circumscribed, and encapsulated mass (5.2 cm) with focal left common femoral vein invasion. Microscopically, the lesion demonstrated poorly-differentiated, oval-to-spindle cells with prominent staghorn vasculature interspersed were focal areas of well-differentiated hyaline cartilage. Immunohistochemical stains showed that the lesional cells were negative for cytokeratin cocktail, EMA, SMA, desmin, S100 protein, SOX10, STAT6, MUC4, MDM2, and ER. Next-generation sequencing (NGS) revealed a HEY1-NCOA2 gene fusion, confirming the diagnosis of extraskeletal mesenchymal chondrosarcoma. Results (if a Case Study enter NA) N/A Conclusion Extraskeletal mesenchymal chondrosarcoma can rarely present as an inguinal soft tissue mass with vascular invasion, mimicking a thrombosed venous aneurysm. Molecular confirmation of HEY1-NCOA2 gene fusion can help confirm the diagnosis in unusual clinical presentations.


Suizo ◽  
2013 ◽  
Vol 28 (6) ◽  
pp. 792-799
Author(s):  
Chihiro KAWAGUCHI ◽  
Masayuki SHO ◽  
Takahiro AKAHORI ◽  
Shoichi KINOSHITA ◽  
Minako NAGAI ◽  
...  

Author(s):  
Saloni Naresh Shah ◽  
Ashok Parameswaran ◽  
Prasanna Kumar Reddy

AbstractExtraskeletal mesenchymal chondrosarcoma (ESMC) metastasizing to the pancreas in isolation is a rare occurrence. We report a 49-year-old gentleman who had undergone excision of an ESMC of the thigh in 2009 and presented with sudden onset abdominal pain and icterus in 2019. Radiological imaging revealed calcified mass of the pancreas with multiple nodules with extension into the adipose tissue. Distal pancreatectomy was performed and the pathology revealed a bimorphic tumor composed of undifferentiated round blue cells with abrupt transition to hyaline cartilage, typical of mesenchymal chondrosarcoma. To the best of our knowledge, there are only seven prior cases of metastatic ESMC of the pancreas in the English literature. Surgical intervention appears to be the preferred modality of treatment for metastatic pancreatic tumors. These patients may have long latency period before metastasizing and seem to have a good survival period post excision.


Author(s):  
Aluisio Rosa Gameiro Filho ◽  
Guilherme Sturzeneker ◽  
Ever Ernesto Caso Rodriguez ◽  
André Maia ◽  
Melina Correia Morales ◽  
...  

Abstract Background To report a case of acute exudative polymorphous paraneoplastic vitelliform maculopathy in a patient with a history of choroidal melanoma, with metastases to the pancreas, liver, and central nervous system. Case presentation A 63-year-old patient, with a history of enucleation of the right eye due to choroidal melanoma, complained of progressive visual loss during a follow-up visit. Fundoscopic examination revealed multiple small areas of serous retinal detachment scattered throughout the posterior pole and ancillary tests confirmed the diagnosis of acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM). Screening for systemic metastases showed pancreatic, hepatic, and central nervous system involvement. Conclusions We describe a rare case of acute exudative polymorphous paraneoplastic vitelliform maculopathy, which should be considered in patients with or without a history of melanoma, who have vitelliform retinal detachments. Nevertheless, no previous reviews of literature have shown a correlation between AEPPVM and pancreatic metastasis.


2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Gabriel A. Molina ◽  
Darwin R. Ramos ◽  
Alberto Yu ◽  
Patricio A. Paute ◽  
Paul S. Llerena ◽  
...  

Endometriosis is a common entity among fertile women which unfortunately manifests through variable symptomatology. Intestinal involvement in endometriosis is quite common and can simulate several diseases such as Crohn’s disease, appendicitis, tubo-ovarian abscess, or malignant tumors. Intestinal obstruction due to endometriosis is rare, and preoperative diagnosis is difficult because the signs and symptoms are nonspecific and can be easily confused. In the case of patients without a history of endometriosis, diagnosis is further complicated. We present a case of a 41-year-old female patient. She presented to the emergency room with complete bowel obstruction and a mass in the cecum. Surgery was decided, and the patient underwent full recovery. Endometriosis was the final diagnosis for the observed condition.


Rare Tumors ◽  
2020 ◽  
Vol 12 ◽  
pp. 203636132098369
Author(s):  
Bouhani Malek ◽  
Sakhri Saida ◽  
Jaidane Olfa ◽  
Kammoun Salma ◽  
Slimene Maher ◽  
...  

Pancreatic metastases are rare, accounting for 2%–3% of pancreatic tumors. The pancreas represents an unusual metastatic site of synovial sarcoma (SS) outside the usual localizations (regional nodes, lung, bone, and liver). The diagnosis is evoked by the personnel medical history of SS and imaging then confirmed by histological examination of the guided pancreatic biopsy. Its therapeutic management is mainly surgical with extensive removal of the lesion. So far only four cases have been reported in the English literature. We reported the case of a male aged 30-year-old who was admitted to our Institute for a local recurrence of SS of the left thigh which was initially treated by surgical excision. The patient underwent a wide surgical excision followed by chemotherapy and radiotherapy. About 15 months later, he experienced a pancreatic metastasis of his SS. He had a caudal splenopancreatectomy with partial resection of the transverse colon followed by chemotherapy. This report highlights the diagnostic difficulties of this rare localization and therapeutic challenge.


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