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Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive neoplasm. The cytological diagnosis of this tumor has only been reported in a few cases. In most of these cases, the diagnosis was made using fine-needle aspiration cytology. Most DSRCTs resemble disseminated carcinomatoses in their clinical manifestation as well as cytomorphologically, even in young-adult patients. These authors report a case of using peritoneal-washing and pleural-effusion ThinPrep cytology to diagnose DSRCT, with extensive glandular differentiation and mucin vacuoles. We found that fibrillary stromal fragment, clinical setting, and adjunctive immunocytochemical staining were most helpful for avoiding misdiagnosis.
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Gastric cancers with microsatellite instabilities (MSI) have been reported to be associated with favorable prognosis. However, the significance of the effect of MSI on the clinicopathological features, as well as its association with mucin phenotype, remains unclear.
MSI status was assessed in 414 cases of gastric cancer using polymerase chain reaction analysis of five microsatellite loci, as recommended by National Cancer Institution criteria. The expression of mucins (MUC5AC, MUC6, MUC2, and CD10) was assessed.
Out of 414 total cases of gastric cancer, 380 (91.7%), 11 (2.7%), and 23 (5.6%) were microsatellite stable (MSS), low-level MSI (MSI-L), and high-level MSI (MSI-H), respectively. Compared to MSS/MSI-L, MSI-H gastric cancers were associated with older age (p=0.010), tumor size (p=0.014), excavated gross (p=0.042), intestinal type (p=0.028), aggressive behaviors (increase of T stage [p=0.009]), perineural invasion [p=0.022], and lymphovascular emboli [p=0.027]). MSI-H gastric cancers were associated with tumor necrosis (p=0.041), tumor-infiltrating lymphocytes (≥2/high power field, p<0.001), expanding growth patterns (p=0.038), gastric predominant mucin phenotypes (p=0.028), and MUC6 expression (p=0.016). Tumor necrosis (≥10% of mass, p=0.031), tumor-infiltrating lymphocytes (p<0.001), intestinal type (p=0.014), and gastric mucin phenotypes (p=0.020) could represent independent features associated with MSI-H gastric cancers. MSI-H intestinal type gastric cancers had a tendency for poor prognosis in univariate analysis (p=0.054) but no association in Cox multivariate analysis (p=0.197).
Our data suggest that MSI-H gastric cancers exhibit distinct aggressive biologic behaviors and a gastric mucin phenotype. This contradicts previous reports that describe MSI-H gastric cancer as being associated with favorable prognosis.
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Mucinous cystadenocarcinoma (MCA) in the breast is a rare neoplasm. There have been 13 cases of primary breast MCA reported. The MCA presents as a large, partially cystic mass in postmenopausal woman with a good prognosis. The microscopic findings resemble those of ovarian, pancreatic, or appendiceal MCA. The aspiration findings showed mucin-containing cell clusters in the background of mucin and necrotic material. The cell clusters had intracytoplasmic mucin displacing atypical nuclei to the periphery. Histologically, the tumor revealed an abundant mucin pool with small floating clusters of mucin-containing tumor cells. There were also small cysts lined by a single layer of tall columnar mucinous cells, resembling those of the uterine endocervix. The cancer cells were positive for mucin (MUC) 5 and negative for MUC2 and MUC6. This mucin profile is different from ordinary mucinous carcinoma and may be a unique characteristic of breast MCA.
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Primary thymic mucinous adenocarcinoma is an extremely rare aggressive subtype of thymic carcinoma. With a review of literatures, only nine cases have been reported up to present. A 36-year-old woman was admitted for further evaluation and treatment of a mediastinal mass. The patient had no medical history of cancer. The clinicoradiological examination disclosed no tumor elsewhere. After the surgical excision of mediastinal mass, it was grossly a round semi-solid mass with mucin-filled cystic areas. Microscopically solid areas showed cords, small nests and dilated glands infiltrating the fibrotic parenchyma, while the cystic areas were lined by mucinous epithelium with tumor cells floating in extracellular-mucin pools. Some cystic walls underwent malignant transformation of the benign thymic epithelium. Immunohistochemically, the tumor cells were positive for cytokeratin (CK) 7, CK20, CD5, and CDX-2, and negative for thyroid transcription factor-1. In conclusion, the mucinous thymic adenocarcinoma should be recognized as a separate histopathological entity and considered in the differential diagnosis of mediastinal carcinomas.
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Urachal mucinous tumor of uncertain malignant potential is very rare and is characterized by a multilocular cyst showing the proliferation of atypical mucin-secreting cells without stromal invasion. As in ovarian and appendiceal borderline tumors, it represents a transitional stage of mucinous carcinogenesis in the urachus. In addition, this tumor may recur locally and develop into pseudomyxoma peritonei. Due to its scarcity and diagnostic challenges, we report a mucinous tumor of uncertain malignant potential arising in the urachus.
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