Histologic, histochemical, immunocytochemical, and ultrastructural features of two cardiac myxomas containing glandular elements are reported. Glandular elements in both cases stained positively with both mucicarmine and periodic acid-!khiff reagent with diastase pretreatment (DPAS). Immunoperoxidase studies demonstrated positivity of the glandular cells for carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA), and keratin. Factor VIII-related antigen (FVIIIAg) was identified only in cells lining vascular spaces. Electron microscopic study of one tumor demonstrated well-formed glands having basement membranes, junctional complexes, and apical secretory granules. These findings indicate the capacity for true epithelial differentiation of cardiac myxomas and have implications both as regards the histologic diagnosis of these tumors and their histogenesis. Cancer 59:1767-1775, 1987. ARDIAC MYXOMA is the most common primary tu-C mor of the heart,',* and is considered by most authors to be a mesenchymal neoplasm and not merely the result of a thrombotic proce~s.',~-~ One characteristic of myxomas which has been cited as evidence for their neo-plastic nature is the occasional presence of histologically distinct gland-like elements within tumors which are otherwise grossly and microscopically typical of m y ~ o m a. ~ Although previous studies of cardiac myxomas have described the light m i c r o s ~ o p i c ~ ~ ~ and ultra~tructural~ features of such glandular elements, studies of the immu-nohistochemical characteristics of these structures have been limited to the demonstration of positivity for Factor VIII-related antigen (FVIIIAg), a finding which has been interpreted as supporting an endothelial origin for these turn or^.^ Although other ultra~tructural~~~ and immuno-histochemical' studies of cardiac myxomas have demonstrated tumors comprising a heterogeneous population of cells with features of endothelium, smooth muscle cells,
The histogenesis of alveolar soft part sarcoma (ASPS) is a subject of continued debate. Although many recent reports suggest a muscle origin, others advocate a neuroendocrine derivation. A tumor in the chest wall of a 16-year-old woman was diagnosed and treated as ASPS. The light microscopic, electron microscopic, and immunohistochemical findings showed features of both ASPS and paraganglioma. In addition, this lesion was positive for antibody to glucagon, a characteristic of neither ASPS nor paraganglioma, although seen in a few gangliocytic paragangliomas. This case demonstrates the need for continued inquiry into the histogenesis of ASPS. Cancer 67:1894-1899,1991. LVEOLAR SOFT PART SARCOMA (ASPS) is a rare ma-A lignant tumor of soft tissue. There has been much speculation concerning its histogenesis. The most widely proposed theories include neural crest origin, as in granular cell tumor, ' paraganglial skeletal muscle origin,'-' and the angioreninoma theory.' We recently encountered an unusual tumor arising in the chest wall deep to the breast of a 16-year-old woman. The tumor had features of both ASPS and paraganglioma and was immunoreactive with antiglucagon antibody, a feature of neither. We present this case and discuss the controversies concerning our knowledge of ASPS. Case ReportA 16-year-old woman had a Ilh-year history of a right breast mass. On examination, a 6.0-cm rubbery, soft, mobile, wellcircumscribed nodule was noted arising from the chest wall deep to the breast, extending from the areola outward along the 10: 30 o'clock axis. There was no skin dimpling, nipple retraction, or palpable axillary adenopathy. Baseline blood values were normal. The patient underwent excision of the mass under local anesthesia. After a diagnosis of ASPS, the patient underwent mastectomy at another institution.
Neoadjuvant chemoradiation is one of the cornerstones of colorectal adenocarcinoma management. This approach can lead to tumor regression in some patients and has shown to be prognostically beneficial. We report a case of rectal neuroendocrine tumor occurring following chemoradiation of a rectal adenocarcinoma. The patient is a 45-year-old man of Asian descent who was found to have a moderately differentiated nonmucinous adenocarcinoma of the rectum during a rectal bleeding work-up, for which he received chemoradiation prior to resection. The resection specimen showed an ulcerating lesion in the rectum at the site of previous adenocarcinoma. Microscopic evaluation revealed nests of well-differentiated neuroendocrine cells. Immunohistochemistry for AE1/AE3, synaptophysin, chromogranin A, S100, melan-A, HMB45, RCC, PSMA, PSAP, CD68, and Ki67 was performed. No histopathologic evidence of residual adenocarcinoma was present in the resection specimen at the location of the original biopsy, indicative of tumor regression. However, a well-differentiated (G1) neuroendocrine tumor was found at this same site. The tumor stained with AE1/AE3 and synaptophysin, and was negative for all other stains. Only 5 similar cases have been reported in the literature, in one case series, which have shown neuroendocrine tumors developing in the context of complete or partial regression of primary rectal adenocarcinoma following chemoradiation. Several case series have shown increased numbers of neuroendocrine cells in post chemoradiationtreated rectal adenocarcinomas. Further study is needed to establish the association between chemoradiation and the development of increased neuroendocrine cells and neuroendocrine tumors in the rectum.
Nasopharyngeal carcinoma (NPC) is an aggressive Epstein Barr virus (EBV) associated carcinoma accounting for 3.7% of upper aerodigestive tract malignancies. At presentation, most patients (60-72%) demonstrate cervical lymphadenopathy, and 5-11% will have clinically apparent distant metastases, with 78% developing distant metastatic disease within 18 months of presentation. The most common sites for distant metastases are bone, liver, lung, and bone marrow. We present a 38-year-old man with a right nasopharyngeal mass, ipsilateral cervical lymphadenopathy, and enlarged periportal lymph node. Neck and chest computed tomography scan revealed a 1.8 cm hypodense mass in the right lateral parapharyngeal space with right posterior cervical adenopathy. Positron emission tomography scan showed increased fludeoxyglucose uptake in the neck and in the porta hepatis region. The patient underwent palpation guided fine needle aspiration biopsy (FNA) from the neck mass, core needle biopsies of the right nasopharyngeal mass, bilateral tonsillectomy, and right neck dissection. The FNA, nasopharyngeal biopsies, and right neck levels 2a, 3 and 4 lymph nodes showed nonkeratinizing NPC, undifferentiated type (WHO type III), which was confirmed by strong nuclear positivity for EBV by in situ hybridization and focal positive immunohistochemical staining for cytokeratins AE1:AE3 and CAM5.2. Negative immunohistochemistry included CK5/6, P63, CK903, EMA, CD45, CD20, CD3, CD4, CD5, CD138, FLI1, MUM1, PAX5, vimentin, desmin, S100, melan A, HMB45, and SMA. Both tonsils and all level 2b lymph nodes were negative for carcinoma. Due to the unusual location, the periportal lymph node was sampled to rule out a second primary tumor. Endoscopic ultrasound guided FNA was performed, which showed metastatic NPC. To the best of our knowledge, this is the first documented case of periportal lymph node metastasis of nasopharyngeal carcinoma.
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