Clinical Reasoning: Longitudinally Extensive Spinal Cord Lesions in a Middle-aged Man

An immunocompetent 47-year-old man presented with a five-month history of progressive lower limb weakness, back pain, sphincter dysfunction, and intermittent fever, suggesting myelopathy in a chronic deteriorating course. A comprehensive analysis comprising of blood tests, neuroimaging, CSF profiling, molecular analysis, and histopathology was performed. Notably, enhanced spinal cord MRI revealed longitudinally extensive intradural-extramedullary lesions involving the cervical, thoracic, and lumbosacral spinal cord, with homogeneous enhancement and spinal cord compression. Serum TPHA and RPR tests were positive. CSF profiling showed pleocytosis, significant protein elevations, hypoglycorrhachia, and positive TPHA test. 18F-FDG-PET/CT indicated slightly increased intraspinal FDG uptake. Spinal cord biopsy further showed small round blue cells in poorly differentiated tissues. Immunostaining was positive for NKX2.2, CD56, CD99, Synaptophysin, and Ki67 (50%). Molecular analysis detected a novel MALAT-CYSLTR1 fusion protein and variants in oncogenic genes including PTCH1, TERT, CREBBP, SPEN, and STK11. The diagnosis of intraspinal extraosseous Ewing’s sarcoma (ES) was confirmed. Briefly, our case details the diagnosis of a patient with intradural-extramedullary ES and highlights the value of spinal cord biopsy in progressive myelopathy of unknown causes.

Challenges of Diagnosing Primary Ewing’s Sarcoma in the Small Intestine of the Elderly: A Case Report

Extraosseous Ewing’s sarcoma (EES) is a malignant tumor that is classified as a rare disease. EES is common in children and adolescents, with a rarer incidence being present in the elderly. ES of the primary intestine is rare, with only a few reports described in the literature. Here we report a case of a 69-year-old male patient who was experiencing abdominal pain for over 3 months. Ultrasonography (US) revealed a solid hypoechoic lesion with multiple irregular necrotic areas in the left lower abdomen close to the dome of the bladder. Contrast-enhanced ultrasonography (CEUS) showed that the lesion exhibited heterogeneous enhancement and quick peripheral enhanced tissue wash-out classifying this mass as malignant. PET–CT showed a high metabolic mass in the lower abdomen, multiple metabolic nodules in the mesentery, considered as a small intestinal stromal tumor with lymph nodes metastasis, and that a diagnosis of lymphoma should be excluded. Esophagogastroduodenoscopy performed at another hospital 1 month prior to CT showed an abnormal density in the pelvic cavity that was considered as a colonic diverticulum with an abscess. The endoscopy showed no obvious occupying lesions. The mass was removed and postoperative pathology confirmed ES of the small intestine. The patient avoided receiving chemotherapy. After 2 months, skull metastasis was diagnosed and surgical intervention was done. His survival was only six months after the second surgery. To our knowledge, our case is the first report of ultrasound and CEUS manifestation of EES in the small intestine in elderly.

Dilemma in diagnosis and management of rare primary pleural Ewing’s sarcoma with synchronous limited metastatic disease

We present a case of a 34-year-old woman who presented with complaints of fever, cough and dyspnoea of 2 months’ duration. On evaluation, she was diagnosed with a rare entity primary pleural Ewing’s sarcoma with synchronous metastases to mediastinal, supraclavicular nodes and single vertebra. Due to the rarity of this entity and lack of treatment guidelines on extraosseous Ewing’s sarcoma, the patient was managed with a combination of multiagent chemotherapy, surgery and radiotherapy as per standard guidelines for skeletal Ewing’s sarcoma. We present this case to discuss differential diagnoses and management dilemmas encountered on the use of local modalities such as surgery and radiotherapy for control of primary and metastatic sites.

Extraosseous Ewing’s Sarcoma: Pictorial Review of Imaging Findings, Differential Diagnosis, and Pathologic Correlation

Extraosseous Ewing’s sarcoma (EES), first described in 1969, is a malignant mesenchymal tumor just like its intraosseous counterpart. Although Ewing’s sarcomas are common bone tumors in young children, EESs are rarer and more commonly found in older children/adults, often carrying a poorer prognosis. We discuss the multimodality imaging features of EES and the differential diagnosis of an aggressive appearing mass in proximity to skeletal structures, with pathologic correlates. This review highlights the need to recognize the variability of radiologic findings in EES such as the presence of hemorrhage, rich vascularity, and cystic or necrotic regions and its imaging similarity to other neoplasms that are closely related pathologically.

Primary pulmonary Ewing’s sarcoma: rare cause of massive hemothorax in a young girl-case report

Background Ewing’s sarcoma is a common malignant bone tumor in children and young adults. Rarely, extra-skeletal soft tissues and visceral organs can also be the site of origin of Ewing’s sarcoma. Primary pulmonary Ewing’s sarcoma is an extremely rare malignancy. Case presentation We report an unusual case of primary pulmonary Ewing’s sarcoma in a 15-year-old girl who initially presented with massive hemothorax. By histopathology evaluation of surgical biopsy specimens, the diagnosis of extraosseous Ewing’s sarcoma was confirmed by both light microscopy and immunohistochemistry. Emergency, open surgery was performed by thoracic surgery at an early stage. After 3 cycles of chemotherapy, the patient was found to be stable at follow-up examination. No more hydrothorax or other symptoms. Conclusions We have described an extremely rare case of primary pulmonary Ewing’s sarcoma with massive hemothorax. The patient underwent surgical resection and postoperative chemotherapy, no sign of recurrence to date as an outcome.

Primary Ewing’s Sarcoma of the Lumbosacral Spine: A Case Report

Ewing's sarcoma is a rare tumor first discovered by James Ewing in 1921. It is more common in bone or skeletal component compared to soft tissue or extraosseous Ewing's sarcoma. Among soft tissue Ewing’s, spinal cord involvement is rarer with only nine cases reported. We report a case of nine-year-old Malay girl who presented with low back pain for two months following a fall with progressive neurological deficits of bilateral lower limb. Magnetic resonance imaging was suggestive of a well-defined margin of intradural extramedullary tumor. With nerve sheath tumor in mind, surgical excision with laminectomy L2-S1 was performed. Intraoperative finding was an extradural mass from L3-L5 with extension to bilateral neuroforamen. Histopathology report defined a round cell tumour of Ewing’s sarcoma from the mass.