Splenic undifferentiated high grade pleomorphic sarcoma of a small size with fatal tumor rupture

Abstract

Primary undifferentiated pleomorphic sarcoma of the spleen is a rare and highly aggressive neoplasm that usually presents with splenomegaly, constitutional symptoms and frequent distant metastases. We report a case of 77-year old male patient with a past history of dissecting aortic aneurysm that developed acute hemorrhagic shock. Aneurysmal rupture was clinically suspected, but the postmortem examination revealed a 25 mm-sized tumor in an atrophic spleen weighing 65 gram with massive retroperitoneal bleeding. Metastases were found in the right renal hilus, the right adrenal gland and femoral bone marrow. Histology of the tumor showed undifferentiated pleomorphic sarcoma. Tumor rupture with fatal bleeding and systemic metastases had occurred despite the small size of the tumor. Tumor size is not a reliable predictor of systemic metastasis or tumor rupture for splenic undifferentiated pleomorphic sarcoma.

Pleomorphic spindle cell sarcoma (PSCS) (formerly known as malignant fibrous histiocytoma, MFH) of the spleen, mimics an atypical haemangioma on99mTc-RBC, CT and Ultrasound: staging with18F-FDG PET/CT

“…While these tumours are now believed to be the most frequently encountered malignant soft tissue tumours, 4 , 5 they are extremely rare in the spleen, with less than 20 cases of splenic MFH reported in the literature. 6 – 9 Splenic MFH occurs in adults aged 40–60 with a slight male predominance, and symptoms such as abdominal pain, splenomegaly, weight loss and fever are common in about 70% of these patients. It is a very aggressive malignancy with high potential of local recurrence and distant metastases, usually to the liver.…”

“…18 F-FDG PET/CT has proven to be useful in the staging of MFH and identification of metastatic disease in unusual locations 12 – 14 and the use of 18 F-FDG PET/CT for staging of aggressive sarcomas including MFH is recommended in the joint RCR and RCP evidence-based guidelines for the use of PET/CT in the United Kingdom (2016); 15 however, to our knowledge, the use of 18 F-FDG PET/CT in the staging of splenic PSCS has not been previously described. Owing to the highly aggressive nature of this splenic malignancy, including two recent reports of spontaneous tumour rupture 6 , 10 timely imaging with PET/CT is crucial for implementation of treatment. Histopathological examination is essential both for confirming the diagnosis of splenic PSCS and for grading and staging of these tumours.…”

Spontaneous splenic artery rupture in a patient with an unclassified malignant spindle cell tumor of the spleen: report of a case

“…In their series, which included 9 patients, the 6-month survival rate was 21% 10 . Patients presenting with spontaneous rupture fared worse 11 – 13 .…”

Splenic Lesions

“…Kaposi sarcoma is a spindle cell neoplasm that has a well-known association with human immunodefi ciency virus (HIV) infection and acquired immunodefi ciency syndrome (AIDS) [ 34 ]. AIDS-related Kaposi's sarcoma has a relatively common association with the liver, but not the spleen.…”

“…Pleomorphic undifferentiated sarcoma, formerly called malignant fi brous histiocytoma [ 34 ], is the most common soft tissue malignant neoplasm in adulthood but is rarely found in the spleen. Less than 15 cases have been reported in the English medical literature as of 2012 [ 35 ].…”