Metanephric stromal tumor (MST) of the kidney is a rare pediatric neoplasm which has rarely been reported in adults. The authors present a case of MST, cellular type, an unreported variant, in a 56-year-old postmenopausal woman. The 9.0-cm tumor was solid, unencapsulated, and well circumscribed, with a firm homogeneous cut surface. The tumor was purely stromal with dense spindle-cell proliferations displaying prominent schwannoma-like architecture, intratumoral angiodysplasia, and entrapped native renal tubules with "onion-skin" tumor collarettes. The tumor stroma expressed CD34, estrogen, and progesterone and did not express desmin, smooth muscle actin, S-100, or TLE-1. Considering the characteristic morphology and immunophenotype, a diagnosis of "metanephric stromal tumor, cellular type," was made. This is an exceptionally rare disease manifestation in an adult patient and the first description of MST with such high stromal cellularity, making this case unique in both clinical and pathological presentation.
Cryptococcosis is infrequent in children and isolated cryptococcal osteomyelitis is rarely encountered. Here we describe a 14-year old patient in remission from T-cell acute lymphoblastic leukemia with osteomyelitis due to Cryptococcus neoformans var. grubii. The patient was effectively treated with antifungal therapy.
Liposarcoma in the context of neurofibromatosis is very rare. To the best of our knowledge, only six cases have been reported until now in the literature. We are presenting this case to underline the possibility of recurrence in the case of retroperitoneal DDLSs despite local tumour resection. Also, although the role of chemotherapy is controversial we decided to start treatment with cisplatin and doxorubicin given the success of chemotherapy in similar case presentations.
INTRODUCTION: Crohn’s disease is an inflammatory bowel disease that may present anywhere along the gastrointestinal tract, spanning from the esophagus to the large intestine. The majority of cases of Crohn’s disease are located in the colon and small intestine, constituting approximately 70% of cases. Rarely will Crohns disease present in the stomach. Here we present a rare case of gastric outlet obstruction secondary to Crohn’s disease of the stomach. CASE DESCRIPTION/METHODS: Patient is a 53 year old female with a medical history of Crohn’s disease who presented to the emergency department complaining of epigastric abdominal pain, nausea and yellow vomiting for three days. Notable laboratory workup included a complete blood count and comprehensive metabolic panel within normal limits. Notable imaging included a computed tomography of the abdomen with contrast which showed a dilated stomach suggestive of gastric outlet obstruction. This was followed by a barium swallow which showed a dilated stomach, delayed gastric emptying, and inflammatory changes in the pyloric channel. An upper esophagogastroduodenoscopy was then done which showed diffuse edematous, ulcerated mucosa in the antrum with pyloric narrowing. Biopsies were taken of this area which showed extensive surface erosion and fresh granulation tissue with transmural chronic inflammation. The option for repeat EGD with balloon dilation or surgical intervention were discussed with the patient, but she opted for conservative measures. This consisted of total parenteral nutrition and treatment with intravenous high dose steroids (Solumedrol 60 mg IV). During her course the patient slowly improved was able to tolerate oral intake. She was then discharged with oral steroids and follow up appointments. DISCUSSION: Crohn’s disease of the stomach is rare occurrence. The first case of gastric crohns disease was reported in 1949. Since then current data suggests that a mere 0.5 to 4% of all Crohns disease cases present with gastric involvement. Diagnosis of gastric crohns disease can be accomplished through biopsy. This would show classic transmural inflammation, which was consistent with the biopsy findings in our patient. Symptoms of gastric Crohn’s disease are varied and can include symptoms of gastric outlet obstruction if the pylorus is involved. This includes nausea, post-prandial vomiting, abdominal pain and bloating. These cases have been successfully treated with endoscopic balloon dilation, Adalimumab, and surgical resection in more severe cases.
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