Bilateral renal metastases from extraskeletal mesenchymal chondrosarcoma of thigh

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.

Extraskeletal mesenchymal chondrosarcoma mimicking a thrombosed venous aneurysm with femoral vein involvement: A case report

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.

Extraskeletal Mesenchymal Chondrosarcoma in Nasal Cavity: A Rare Case Report

Extraskeletal mesenchymal chondrosarcoma (ESMC) originate from the nasal cavity have rarely been reported, especially its imaging features, which makes the preoperative diagnosis difficult. Here, we report the clinical, computed tomography, and magnetic resonance imaging features of a 60-year-old female patient with pathologically confirmed ESMC in the nasal cavity to help provide more reference for diagnosis before operation. Extraskeletal mesenchymal chondrosarcoma in the nasal cavity demonstrates typical imaging features, such as mesh-like enhancement, calcification, hemorrhage, necrosis, cystic degeneration, and so on.

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

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.

Metastatic Extraskeletal Mesenchymal Chondrosarcoma of the Pancreas: Report of an Unusual Case with Review of Literature

Extraskeletal 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.

Radiological Features of the Intracranial Extraskeletal Mesenchymal Chondrosarcoma: A Report of Two Cases Insight Into the Literature Review

Background: Mesenchymal chondrosarcomas are the most malignant form of chondrosarcomas. They have mostly affected bones. Rarely, these tumors can be intracranial extraskeletal (IEMC) that originates from the meninges or parenchyma. Methods and Materials: We presented two IEMC patients who were treated at our institutions and followed up for the long-term. To understand the radiological features of IEMC, we conducted a systematic literature review for previously reported series and cases of IEMCs. Results: We surgically treated two young males with IEMC initially diagnosed at their age of 18 and 20 years. The patients initially treated with gross total resection (GTR) and GTR followed by radiotherapy, and followed-up for 218 and 73 months, respectively. With both patients, we obtained 83 reported IEMC patients from the literature. Among them, only 30 cases were reported with their radiological MRI details. The mean age of the reported cases was 24.5±16.0 years (2 months–71 years). Female predominance was 54.2%. The mean progression-free and overall survivals were 27.9 and 39.0 months, respectively. Most IEMCs showed a partially calcified mass on roentgenography and a highly vascular mass on angiography. On T1WIs, IEMCs almost show hypo- to isointensity and intense heterogeneous enhancement after administering a contrast substance. On T2WIs, IEMCs show iso- to hyperintensity.Conclusions: IEMCs usually show dural attachment without a net dural tail sign and a well-identified brimmed vascular nodule on TOF-MRA. This nodule appears as a prominent blooming on SWI. TOF-MRA and SWI images can help in the radiological diagnosis of IEMCs.

Mesenchymal Chondrosarcoma of the Brain with Metastasis: A Case Report with Literature Review

Mesenchymal chondrosarcoma of the brain is one of the rarest tumors with dismal prognosis. A 26-year-old man presented with headache, vomiting, and diplopia. On evaluation, a moderately enhancing extra-axial lesion was seen in right temporal region involving right greater wing of sphenoid, adjacent right maxillary sinus, and lateral wall of right orbit. Patient underwent magnetic resonance imaging of the spine and computed tomography (CT) of the chest for possible metastasis which showed lesion in lumbar vertebrae and left lung. Patient was planned for CT-guided lung biopsy which proved inconclusive. The patient was further planned for craniotomy and underwent craniotomy with microscopic excision of the mass. Histoimmunochemistry was suggestive of extraskeletal mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma of the brain is highly aggressive tumors which are difficult to differentiate radiologically. Radical excision followed by chemoradiotherapy is optimal treatment of choice.

Extraskeletal Mesenchymal Chondrosarcoma

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.