Can you diagnose this patient case, a giant splenic mass, presented by a SERMO pathologist?
A 45 year old Caucasian woman with travel to the Middle East presents with persistent fever. Chest x-ray obtained showed left upper quadrant calcified structure, prompting further imaging. CT and MRI revealed large splenic lesions concerning for Echinoccocus. Patient was treated empirically with albendazole per ID without improvement. Splenectomy was performed.
This is a case of primary “Pleomorphic Undifferentiated Sarcoma” aka ” Malignant fibrous histiocytoma “of the spleen. MFH is the most common soft tissue sarcoma mainly occurring in the extremities (70%), followed by the abdominal or retroperitoneum (16%).The origin of MFH is still controversial. Primary MFH of the spleen is extremely rare, although the incidence of MFH of the spleen remains undetermined. Since the first description of primary splenic MFH reported by Govoni et al.6 in 1982, only 13 cases have been reported in the English language literature in Medline.
The incidence of splenic MFH remains undetermined due to limited data on a rare malignancy. The clinical symptoms of primary splenic MFH are usually nonspecific, but the characteristics such as abdominal pain, fever, weight loss, and splenomegaly are prominent features. Splenectomy including laparoscopic splenectomy with histological negative margins is the treatment of choice. Chemotherapy and radiotherapy are other therapeutic modalities for patients with metastasis, but the therapeutic effect is poor.