Anti-TIF1γ-Dermatomyositis and Sjögren's Syndrome As the Inaugural Presentation for Rectal Cancer.

Dermatomyositis (DM) is an inflammatory myopathy often paraneoplastic in nature. Patients have characteristic cutaneous findings and possible muscle involvement. In the latter, muscle enzymes are elevated, and the electromyogram shows varied changes. Muscle or skin biopsy and myositis-specific antibodies confirm the diagnosis. Here, we report the case of an 86-year-old woman with cutaneous lesions, proximal weakness, and sicca symptoms. Muscle enzymes and electromyogram were normal. Antinuclear antibodies were elevated, and anti-TIF1γ and anti-Ro52 antibodies were positive. Muscle biopsy was compatible with the diagnosis of DM, and salivary gland biopsy confirmed Sjögren's syndrome. Malignancy investigation identified a rectal cancer, which was resected. This case illustrates a rare form of cancer presentation - anti-TIF1γ DM with normal muscle enzymes and electromyogram and concomitant secondary Sjögren's syndrome. Malignancy screening and multidisciplinary management were crucial to a successful approach.

Acute Pyelonephritis and an Incidental Diagnosis.

Gastrointestinal stromal tumors (GISTs) arise from the gastrointestinal tract. In rare cases, extra-gastrointestinal stromal tumors (EGISTs) occur in the omentum, mesentery, et cetera. They are mostly asymptomatic or have unspecific symptoms. Risk stratification classification systems are based on tumor size, mitotic rate, location, and perforation. The gold standard for diagnosis is a computed tomography (CT) scan. Ultrasound/CT-guided percutaneous biopsy allows histopathology and immunochemistry results (most stain positive for CD117 (c-KIT), CD34, and/or DOG1). Mutational analysis (most are in proto-oncogene c-KIT and platelet-derived growth factor receptor A (PDGFRA)) determines appropriate therapy. Surgical resection is the gold standard of treatment, with adjuvant and neoadjuvant molecular-targeted therapies depending on recurrence risk and mutations. This report describes a rare case of GIST (omentum EGIST) with a rare presentation (acute pyelonephritis) in a 67-year-old woman. Abdominal examination showed tenderness and a positive Murphy sign on the left side. Blood analysis presented microcytic hypochromic anemia, aggravated renal function, leukocytosis, and increased C-reactive protein. Abdominal CT revealed a heterogeneous abdominal mass, and a CT-guided biopsy showed epithelioid cells positive for CD117 and DOG1, which is compatible with a GIST. The patient underwent surgery that determined the GIST's origin from the greater omentum. Histology revealed an epithelioid GIST with large dimensions and a high histologic grade. Genetic testing detected a variant in the PDGFRA gene. With a high risk of progression, the patient received a three-year course of imatinib.