Hemoperitoneum is a rare and potentially life-threatening complication of GIST. We reported a 54-year-old man who developed disseminated intra-abdominal recurrence from a previously resected gastrointestinal stromal tumour (GIST) of the small bowel, and the patient presented with hemoperitoneum. Emergent debulking surgery was performed. A high dose imatinib was prescribed. Despite the presence of residual disease, the patient was well clinically 8 months after the operation. Even though, there is no evidence to support the routine use of debulking surgery in the management of GIST. In our patient, disease progression after second line targeted therapy and the absence of alternative treatment options for spontaneous rupture and hemoperitoneum prompted us to treat the patient aggressively. Resection of the ruptured GIST was carried out for control of bleeding and to prevent recurrent bleeding in this patient with good surgical risks. During the treatment decision-making, the patient's general condition, the risk of surgery and the extent of dissemination were taken into consideration. In this patient who presented with spontaneous rupture of a small intestinal GIST, the novel use of targeted therapy and aggressive surgical treatment produced reasonably good survival outcome.
HighlightsGiant epithelial splenic cyst is rare and splenectomy is the treatment.Auto-transplantation of ectopic splenic tissues during splenectomy may create diagnostic confusion when subsequent endoscopy or CT scan is performed years after the operation.99mTechnetium sulfur colloid scintigraphy is a useful adjunct and should be considered in doubtful cases.
The majority of head and neck cancer are of epithelial origin and lymphoma. Sarcomas of the head and neck are rare (5%). 1 Angiomatoid fibrous histiocytoma (AFH) is a rare type of sarcoma with low-grade malignancy that usually presents in children or young adults. 2,3 Enzinger first described its features and named it angiomatoid malignant fibrous histiocytoma (MFH) in 1979. 2 It was later named AFH because of its slow growth and low risk of metastasis. 3 AFH mainly occurs in extremities (60-90%) and trunk (20-28%). 4,5 Only 5-7% of AFH is found in the head and neck. 4,5 We report the case of a 28-year-old woman who presented with an AFH in the neck that was successfully excised.A 28-year-old woman suffered from left lower cervical mass for 2 years. The left cervical mass increased in size gradually. She had no history of trauma, malignancy, acute or chronic infection. The left cervical mass was 5 cm in size, soft and nontender. A neck lipoma was suspected clinically. During surgery, a circumscribed, multilobulated soft tissue mass was seen. The cut surface of the tumour showed pale grayish solid tissue with multiple intervening variable-sized, blood-filled cystic spaces. The tumour was excised completely. Histopathology showed a soft tissue mass that consisted of short spindle cells with indistinct cell borders and mild nuclear pleomorphism. There was a dense fibrous shell at the periphery of the lesion, with dense infiltration by lymphocytes and many plasma cells. Focal areas of cystic change filled with blood were present. Pathology was consistent with an AFH (Fig. 1). The postoperative course was uneventful. No adjuvant treatment was given. She was disease free on follow up 1 year after operation.AFH is a rare type of sarcoma with low-grade malignancy that usually presents in children or young adults. 2,3 Clinically, it presents as a painless soft tissue mass in the subcutaneous or deep dermal layer of the body and is often mistaken as a haematoma or a haemagioma. In our case, the preoperative diagnosis was a lipoma. Accurate preoperative diagnosis of AFH is difficult. Magnetic resonance imaging and computed tomography scan show the nonspecific finding of fluid-fluid levels within the cystic components of the tumour, which indicates intralesional haemorrhage. This finding may also occur in MFH, haematoma or soft tissue haemagioma. The diagnosis of AFH is based on histopathology study. 2 The four distinctive histopathological features for the diagnosis of AFH are: (i) a fibrous pseudocapsule; (ii) a dense lymphoplasmacytic response; (iii) focal areas of cystic change filled with blood; and (iv) proliferation of spindled or round cells. AFH should be differentiated from the conventional MFH. 4,5 MFH is an aggressive high-grade sarcoma that usually presents in the elderly. It requires a radical surgical resection with generous peripheral and deep margins in all three dimensions. Adjuvant radiotherapy or chemotherapy is needed in selected patients. AFH is a low-grade sarcoma that usually presents in children and ...
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