Scleroderma renal crisis in a case of mixed connective tissue disease

Mixed connective tissue disease (MCTD) is an overlap syndrome first defined in 1972 by Sharp et al. In this original study, the portrait emerged of a connective tissue disorder sharing features of systemic lupus erythematosus, systemic sclerosis (scleroderma) and polymyositis. Scleroderma renal crisis (SRC) is an extremely infrequent but serious complication that can occur in MCTD. The histologic picture of SRC is that of a thrombotic micro-angiopathic process. Renal biopsy plays an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in MCTD patients, helping to predict the clinical outcome and optimizing patient management. We herewith report a rare case of SRC in a patient with MCTD and review the relevant literature

Pigmented hepatocellular adenoma with complete CD34 immunostaining pattern: A diagnostic dilemma

WHO defines hepatocellular adenoma (HCA) as a benign tumor composed of cells closely resembling normal hepatocytes, which are arranged in plates separated by sinusoids. It is more common in women. The present concerns a 41 years female who was found to have a mass lesion in liver on ultrasound while undergoing routine evaluation for dyspepsia. Computed tomography scan of abdomen showed 10 × 8 cm lesion in liver. Extended left hepatectomy was performed. Grossly hepatic cut surface showed circumscribed tumor with dark gray or black color. Microscopy revealed hepatocellular adenoma with abundant Dubin Johnson like pigment deposition. CD34 immunostaining showed complete sinusoidal pattern. We labeled the tumor as pigmented hepatic adenoma with complete CD34 staining pattern. To the best of author′s knowledge only eight cases of pigmented hepatocellular adenoma are described in world literature

Hepatic metastasis with heterologous rhabdomyoblastic differentiation in a patient with gastrointestinal stromal tumor treated with imatinib

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the intestinal tract. In patients with locally advanced and/or metastatic GIST, the introduction of tyrosine kinase inhibitor, imatinib mesylate, has transformed the management of this previously untreatable neoplasm into a treatable entity. Approximately 80% of advanced metastatic GISTs respond to imatinib treatment. However, the majority of patients suffer disease progression at a median of 2 years due to drug resistance. Generally progressing GISTs retain their typical morphology. Herein, we report an extremely rare case of progressive metastatic GIST with heterologous rhabdomyoblastic differentiation after, imatinib mesylate treatment. We also review the relevant literature

Mycophenolate mofetil-induced colitis with graft versus host disease-like features in a liver transplant recipient

Mycophenolate mofetil (MMF) is a commonly used immunosuppressive drug in the management of transplant recipients. Gastrointestinal (GI) toxicity (diarrhea) is the most frequently reported adverse event in MMF-treated transplant patients. MMF-induced Graft versus Host Disease has rarely been reported in literature. We report a case of MMF-induced colitis with Graft versus Host Disease-like features, to highlight the importance of high clinical suspicion for its diagnosis, and that appropriate management in such a setting can reduce morbidity and mortality. We also review the relevant literature

Primary spinal melanoma of the cervical leptomeninges: Report of a case with brief review of literature

Central nervous system primary malignant melanoma accounts for approximately 1% of all melanomas. Primary spinal melanomas are even more unusual. We report a patient with primary spinal melanoma of the cervical leptomeninges. The histology of the tumor showed tumor cells arranged in sheets, ill-defined fascicles and nests and displayed a moderate grade of cellular and nuclear pleomorphism and mitoses with abundant pigment in the cytoplasm. The tumor cells were immunoreactive for HMB-45, and for S-100

Meningioma with hemorrhagic onset: Two case reports

Haemorrhage is a rare complication of meningiomas that can occur spontaneously, after embolization, stereotactic radiation and perioperatively. Our first case was a 16 year old male, admitted with spastic quadriparesis, and retention of urine. Magnetic Resonance Imaging (MRI) revealed anteriorly placed cervical intradural extramedullary mass. Patient underwent emergency surgery following sudden worsening of neurological symptoms and intratumoral bleed was noted peroperatively. Tumor was labeled as angiomatous meningioma with hemorrhage. The second case was of a 45 year female who presented with history of sudden onset weakness in right upper and lower limb followed by unconsciousness. MRI revealed heterogeneous lesion in left parasagittal area with intratumoral bleed. Left frontal craniotomy with tumour decompression was performed. Tumour was labelled as meningothelial meningioma with haemorrhage. Meningiomas with hemorrhagic onset remain rare, and pathophysiology is still incompletely understood. Prevention and outcome of intratumoral haemorrhage highly depends on early diagnosis and adequate treatment

Aggressive Variant of Hepatic Epstein–Barr Virus-Associated Inflammatory Pseudotumor-like Follicular Dendritic Cell Sarcoma with PD-L1 and SSTR2a Expression

An aggressive Epstein–Barr virus (EBV)-associated inflammatory pseudotumor-like follicular dendritic cell (IPT-like FDC) sarcoma is reported in an adult female. The patient developed multifocal recurrence and passed away 13 months after the initial surgical resection. A bright field microscopic examination of the tumor demonstrated a classical growth pattern and the diffuse expression of Programmed death ligand 1 (PD-L1) and somatostatin receptor 2a (SSTR2a)