Gastric Desmoid Fibromatosis - Report of a Rare Mimic of Gastrointestinal Stromal Tumor.

Desmoid fibromatosis (DF) involving the gastrointestinal tract is extremely rare. Its intramural location and occasional expansile growth pattern within the bowel wall may mimic a gastrointestinal stromal tumor (GIST). Due to the different disease behaviors and management, it is important to make a correct diagnosis before further treatment. We present an extremely rare case of a gastric DF that on imaging appeared as a discrete intramural mass mimicking a GIST and that was preoperatively correctly diagnosed as a DF based on its cytomorphologic, immunohistochemical, and molecular profiles. The patient is a 71-year-old female who presented with dysphagia and unintentional weight loss. A mass was identified at the gastric fundus. Endoscopic ultrasound-guided fine-needle aspirate (FNA) and biopsy (FNB) were performed. The FNA showed a few small aggregates of cytologically bland spindle-shaped cells with elongated nuclei. The FNB yielded small fragments of tissue composed of bland spindle cells demonstrating nuclear and cytoplasmic immunostain for ß-catenin and focal stain for smooth muscle actin (SMA) and desmin. CD117, DOG1, CD34, caldesmon, S100, cytokeratin AE1/AE3, signal transducer and activator of transcription 6 (STAT6), MUC4, progesterone receptor (PR), and anaplastic lymphoma kinase (ALK) were negative, and MIB-1 showed a very low proliferation activity index. Molecular studies performed by targeted next-generation sequencing showed activating mutations in CTNNB1. These results excluded a GIST and confirmed the diagnosis of a gastric DF. Although it is very rare, DF must be included in the differential diagnosis of discrete intramural gastric spindle cell lesions. A definitive diagnosis can be made preoperatively if enough lesional material is available for appropriate immunohistochemical and molecular studies.

Talaromycosis in a Patient on Nintedanib for Interstitial Lung Disease.

Talaromycosis is a fungal infection caused by Talaromyces sp. that is predominantly prevalent in patients with acquired immunodeficiency syndrome in the United States. It is also rarely seen in other individuals who are otherwise immunosuppressed. With the advent of immunotherapy and increasing usage of these novel agents in treating several conditions, the prevalence of talaromycosis may increase, especially in people from endemic regions who might harbor a dormant infection. Clinical presentation is non-specific with respiratory symptoms such as shortness of breath, cough, or even fever that can delay the diagnosis. Little is known about the exact pathogenesis of the condition, and management is largely based on anecdotal evidence and small-sized studies. We present the case of an individual on nintedanib, a tyrosine kinase inhibitor that blocks fibroblast growth factor receptor and used for the treatment of interstitial lung disease, who was diagnosed with talaromycosis.

Secondary Immune Thrombocytopenic Purpura in Renal Cell Cancer: A Paraneoplastic Syndrome.

Secondary immune thrombocytopenic purpura (ITP) as a paraneoplastic syndrome has been reported in literature. It is commonly associated with chronic lymphocytic leukemia (CLL) and hodgkins lymphoma. Its association with solid malignancies, especially renal cell cancer is rare, with only a few documented case reports. Treatment usually consisted of targeting the underlying malignancy or utilization of steroid and intravenous immunoglobulin (IVIG) to improve thrombocytopenia. Here, we describe a case of a 75-year-old man with renal cell cancer (RCC), who presented with secondary thrombocytopenia treated with steroid and IVIG. It is important to keep RCC in differential diagnosis for causes for secondary ITP as this impacts treatment.