Bullous systemic lupus erythematosus in a child responding to dapsone.

Bullous systemic lupus erythematosus is a subepidermal blistering disorder that primarily affects young women and only rarely occurs in children. We report a case of bullous systemic lupus erythematosus refractory to corticosteroid therapy in a 12-year-old boy who was successfully treated with oral dapsone.

Ureteric bud remnant with renal agenesis.

INTRODUCTION: Ureteric bud remnant with renal agenesis is a rare disorder when urogenic system develops. Because of no obvious symptoms, it is usually explored incidentally. CASE PRESENTATION: A 41-year-old male presented with intermittent discharge of turbid fluid from his scrotum. A pinhole was noted in his left scrotum, and an infectious sinus or fistula was impressed. After serial studies, computed tomography revealed agenesis of his left kidney and a cystic lesion over his left scrotum. He underwent resection of the infectious sinus. Near the tail of the sinus, a connection was found to a channel-like structure. Contrast medium was injected which showed a blind end of this channel-like structure. The tube was ligated, and the cut end was sent for surgical pathology, which confirmed a left ureteric bud remnant. CONCLUSION: Complete imaging studies make this diagnosis clearly.

Primary lymphohistiocytic variant of anaplastic large cell lymphoma of the stomach.

Here, we report an unusual case of gastric anaplastic large cell lymphoma (ALCL), lymphohistiocytic variant, in a 70-year-old female patient who presented with epigastric pain, tarry stool and body weight loss. Endoscopic and imaging findings revealed a Bormann type II tumor in the stomach with perigastric lymphadenopathy and multiple tumor nodules in the liver. Total gastrectomy and liver biopsy were performed. Histologically, both gastric and hepatic tumors demonstrated anaplastic large neoplastic cells scattered among numerous reactive histiocytes. Immunostaining of these tumor cells reacted positively for CD30, CD3, CD45 RO/UCHL1, and negatively for epithelial membrane antigen, CD68, lysozyme, CD15, CD79a, CD138, PAX5 and anaplastic lymphoma kinase. Both the morphologic and immunophenotypic findings supported the diagnosis of gastric ALCL of lymphohistiocytic variant with liver metastasis. This patient then received chemotherapy and was still alive after 17 months of follow-up, without evidence of residual disease.

Combined primary neuroendocrine carcinoma and hepatocellular carcinoma of the liver.

We report a unique case of combined primary neuroendocrine carcinoma (NEC) and hepatocellular carcinoma (HCC) of the liver in a 65-year-old male patient. The patient underwent segmental resection of the liver and regional lymph node dissection for a tumor mass that measured 7.5 cm in diameter in the right lobe, with regional lymphadenopathy. Histologically, the hepatic tumor was composed of predominantly small-cell NEC, but admixed with a small island of moderately differentiated HCC. We speculate that the NEC originated from a poorly differentiated tumor clone of an HCC that underwent neuroendocrine differentiation, and that this tumor was now at the end stage of the transitional period from HCC to NEC, based on the small amount of disappearing HCC. Ki-67 and p53 expression were higher in the NEC than in the HCC, and the lymph nodes showed only metastatic NEC. Therefore, this kind of tumor had a more aggressive clinical course in accordance with being an NEC rather than a conventional HCC. Three months after operation, the patient had multiple recurrent tumor nodules within the liver, spreading the metastasis to the adrenal glands and para-aortic lymph nodes. The patient died 1 year after operation.

Spinal atypical teratoid/rhabdoid tumor in a 7-year-old boy.

Reported herein is an unusual case of atypical teratoid/ rhabdoid tumor (AT/RT) of the lumbar spine with an intradural extramedullary location in a 7-year-old boy. Histologically, this tumor contained rhabdoid cells, pale cells, and sickle-shaped embracing cells without primitive neuroectodermal tumor (PNET), mesenchymal or epithelial components. Immunohistochemical staining showed that these tumor cells react positively for epithelial membrane antigen (EMA), vimentin, cytokeratin (AE1/AE3), CD99 and neurofilament protein, but negatively for INI1 antibody. Chromosome 22q deletion was demonstrated on fluorescence in situ hybridization.