Douglas J. Grider

Benign Glandular Schwannoma With Ancient Change.

Benign glandular schwannomas are rare and should be distinguished from malignant peripheral nerve sheath tumors with similar divergent tissue differentiation. The authors present a benign glandular schwannoma with ancient change that developed in the subcutis of a 46-year-old mans posterior calf. He lacked stigmata of neurofibromatosis type 1 (NF1). The glandular elements stained positively for epithelial membrane antigen and pancytokeratin. The spindled cells stained positively for SOX10 and S100 protein, supporting schwannian (neural crest) differentiation. The tumors location and histopathology suggest that the pathogenesis stems from entrapment of sweat glands. Finally, it must be recognized that ancient change may mimic malignancy in these neoplasms as the malignant counterparts have a greater association with NF1 and a poorer prognosis.

Metastatic basal cell carcinoma with loss of p63 and mismatch repair proteins

BCC: basal cell carcinoma HH: hedgehog IHC: immunohistochemistry mBCC: metastatic basal cell carcinoma SUFU: suppressor of fused INTRODUCTION Basal cell carcinoma (BCC) represents nearly 80% of nonmelanoma primary skin malignancies in the United States. However, metastatic BCC (mBCC) is exceedingly rare, difficult to treat, and associated with high mortality, yet it’s easily preventable. We report a case of mBCC in a patient with a 15-year history of a neglected primary BCC on the left upper extremity. Mismatch repair protein expression was lost in the primary tumor and metastatic deposits. In addition, p63 expression, which was present in the primary BCC, was absent in the metastatic tumor cells.

Primary tracheal hyalinizing clear cell carcinoma

Hyalinizing clear cell carcinomas (HCCC), now referred to as clear cell carcinomas (CCC) by the World Health Organization (WHO), are rare tumors usually arising from the salivary glands of the head and neck. We present the first case of a CCC originating from the trachea. A 66 year-old woman with history of hypertension, former smoker, presented to the emergency department reporting worsening shortness of breath. Computed chest tomography revealed a polypoid mass arising from the left posterior-lateral wall of the trachea. The patient developed worsening respiratory failure requiring mechanical ventilation. Bronchoscopy was completed which relieved a malignant airway obstruction and pathology revealed nests of cells with monomorphic nuclei and clear cytoplasm set in a hyalinized stroma. Using break apart probe fluorescence in situ hybridization testing the tissue revealed the presence of a recurring translocation of the Ewing sarcoma (EWSR1) gene, confirming the diagnosis. CCC is a rare primary airway tumor. CCC was first reported to originate in the lung in 2015. We present the first case of CCC arising from the trachea.

Tuberculous Enteritis Presenting as Acute Appendicitis and Perirectal Abscess

Mycobacterium tuberculosis has a wide variety of presentations. A rare occurrence is gastrointestinal tuberculosis. It may occur anywhere along the alimentary canal but usually occurs in the ileocecum with rare involvement of the appendix.

Diffuse Gastric Ganglioneuromatosis: Novel Presentation of PTEN Hamartoma Syndrome—Case Report and Review of Gastric Ganglioneuromatous Proliferations and a Novel PTEN Gene Mutation

Gastrointestinal ganglioneuromatous proliferations are rare, most often found in the colon, and are three types: polypoid ganglioneuromas, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. We present a case of diffuse ganglioneuromatosis in the posterior gastric wall in a nine-year-old female. To our knowledge, this is the first reported case of diffuse ganglioneuromatosis located in the stomach. Only six cases of gastric ganglioneuromatous proliferations have previously been reported, two in English and none were diffuse ganglioneuromatosis. A diagnosis of diffuse ganglioneuromatosis is relevant for patient care because, unlike sporadic polypoid ganglioneuromas or ganglioneuromatous polyposis, most are syndromic. Diffuse ganglioneuromatosis is commonly associated with neurofibromatosis type 1, multiple endocrine neoplasia type 2b, and Cowden Syndrome, one of the phenotypes of PTEN hamartoma tumor syndrome. The patient had the noted gastric diffuse ganglioneuromatosis, as well as other major and minor criteria for Cowden syndrome. Genetic testing revealed a novel frameshift mutation in the PTEN gene in the patient, her father, paternal aunt, and the aunts son who is a paternal first cousin of the patient.

A Rare Cause of Colonic Stricture

68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 Question: A 57-year-old man presented to our hospital with a week of generalized weakness and abdominal pain. Relevant medications included diclofenac 75 mg twice daily, aspirin 81 mg, and clopidogrel 75 mg/d. Vital signs were normal. Physical examination showed mild diffuse abdominal tenderness. Admission blood work revealed a hemoglobin of 8.8 g/dL, decreased from a baseline hemoglobin of 12 g/dL. The patient did not have overt gastrointestinal bleeding, but tested positive for fecal occult blood. A computed tomography scan demonstrated luminal narrowing at the hepatic flexure without bowel wall thickening or obstruction (Figure A). Esophagogastroduodenoscopy was normal. Colonoscopy revealed a circumferential stricture in the right colon with an estimated diameter of 8 mm (Figure B). Biopsies of the stricture showed significant lamina propria fibrosis, eosinophilic infiltration, and mild crypt distortion (Figure C). What was the diagnosis? Look on page 000 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. 95 96 97 98 99 100 Conflicts of interest The authors disclose no conflicts.