Sclerosing rhabdomyosarcoma is a unique rhabdomyosarcoma variant, characterized by a prominent hyalinizing matrix. A notable pitfall is the potential for the unusual matrix and often pseudovascular growth pattern of this lesion to lead to confusion with other sarcoma types, including osteosarcoma, chondrosarcoma, and angiosarcoma. Here we report a case of sclerosing rhabdomyosarcoma arising in a 40-year old male. The tumor was centered in the pterygomaxillary fossa with extensive infiltration into adjacent structures. Fine needle aspiration yielded a preliminary diagnosis of high-grade pleomorphic undifferentiated sarcoma, for which he received neoadjuvant chemotherapy and surgical resection. Microscopic examination showed a malignant spindled to round cell neoplasm with prominent osteoid-like, hyaline stroma. Focal rhabdomyoblastic differentiation and diffuse immunoreactivity for desmin and myogenin aided in diagnosis. Nineteen months status post primary resection, the patient expired with multiple lung and bony metastases. Among 39 cases reported thus far (including the present case), there is a broad age range (0.3-79 years), with an average age at presentation of 27 years. The most commonly involved sites are the extremities (n = 19) and head and neck (n = 15). Most cases have been treated by resection, often combined with radiation and/or chemotherapy. Out of 31 cases with follow-up information provided, 6 patients developed local recurrence, 7 patients developed regional or distant metastasis, and 5 patients died of disease. Herein we discuss the ongoing controversy regarding how sclerosing rhabdomyosarcoma might best fit into existing rhabdomyosarcoma classification schemes, based upon current clinicopathologic and molecular genetic evidence.

No abstract

Salivary duct carcinoma is an aggressive tumor, morphologically similar to high-grade ductal adenocarcinomas of the breast. Although most commonly involving the major salivary glands, approximately 10% of salivary duct carcinomas arise from the minor glands of the palate and oral cavity, with rare isolated cases reported as primary to the larynx, sinuses, and mandible. We present a case of a 53-year-old man with ear discomfort and left facial paresis. He had a 2-year history of chronic middle ear pain and presumed mastoiditis; prior biopsy of the auditory canal showed extensive granulation tissue and epithelial fragments suggestive of a middle ear adenoma. At the time of his most recent presentation, no lesions were radiographically seen within the parotid or submandibular glands. A focused MRI showed 2 foci in the left petrous apex that were consistent with cholesterol granulomas but no welldefined mass to correspond with the biopsy diagnosis of middle ear adenoma. Microscopically, the H&E-stained slides contained many oncocytic glands with a papillary and cribriform growth pattern and focal necrosis. Cytologically, the cells were enlarged with mild to moderate nuclear pleomorphism and scattered mitoses. Histochemical and immunohistochemical stains were performed on the paraffin embedded resection specimen. Mucicarmine stained intraluminal mucin. Cytokeratins (AE1/AE3, CAM5.2), CEA, androgen receptor, and GATA3 highlighted cells of interest. Her2 had weak membranous positivity. P63 marked rare abluminal cells and Ki67 (MIB-1) was <5%. The lesion was negative for chromogranin, synaptophysin, NSE, CK5, CK20, S100, GFAP, and TTF-1. Given the distinct staining pattern and histomorphology, the diagnosis was consistent with salivary duct carcinoma. To our knowledge, following a thorough review of current literature, this is the first case of salivary duct carcinoma to originate in the middle ear.

Gastric adenomas are neoplastic lesions, representing up to 10% of all gastric polyps (Endoscopy 1994; 26:659Y665). Three epithelial subtypes are currently recognized, each carrying unique clinical implications. Intestinal-type gastric adenomas are the most common subtype and by definition contain foci of intestinal type epithelium with acidic intestinal mucins (Mod Pathol 2003; 16:786Y795). They commonly arise in a background of atrophic gastritis with intestinal metaplasia. Foci of high-grade dysplasia and intramucosal or invasive adenocarcinoma are often present. Foveolar-type gastric adenomas are the second most common type of adenoma. Although also dysplastic, they typically have a less aggressive behavior and are rarely associated with high-grade dysplasia or adenocarcinoma. Characterized by gastric epithelium with neutral foveolar mucins, foveolar-type gastric adenomas generally arise in either a background of unremarkable mucosa or familial adenomatous polyposis syndrome (Am J Surg Pathol 2002; 26:1276Y1285). Pyloric gland adenomas are the third most prevalent neoplastic gastric polyp (Virchows Arch 2003; 442:317Y321). They resemble the deep mucous glands found near the pylorus with a remarkably bland appearance. However, similar to intestinal-type adenomas, pyloric gland adenomas are also typically associated with a more aggressive clinical course and arise in a background of atrophic gastritis (Virchows Arch 2010; 457:529Y536). As the awareness of pyloric gland adenoma increases, the relative frequencies of these entities are subject to change.(Pathol Case Rev 2013;18: 70Y74) CASE REPORTA 79-year-old white man with a history of gastroesophageal reflux disease, hiatal hernia, and Barrett esophagus was referred for routine Barrett screening. The endoscopy demonstrated a flat gastric polyp (Fig. 1) measuring 3 cm in length and 0.5 cm in width in the proximal portion of the stomach. An endoscopic mucosal resection (EMR) was performed.Histological examination revealed packed pyloric glandtype tubules with a single layer of cuboidal epithelial cells containing round nuclei and ground-glass appearing eosinophilic cytoplasm. The glands had a markedly bland appearance without cytologic atypia. Some of the glands were dilated. The background fundic glands showed prominent parietal cells with lingular projections to the lumen, consistent with proton pump inhibitor effect. No other obvious abnormalities were appreciated (Fig. 2). A diagnosis of pyloric gland adenoma was made. Surgical margins were clear. CASE REVIEW 70 www.pathologycasereviews.com FIGURE 6. Foveolar-type adenomas are composed of dysplastic gastric foveolar epithelium, consisting of columnar mucosal cells with apical mucin caps. Dysplasia is typically concentrated at the surface of the polyp and is evidenced by the cytologic atypia.

No abstract