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Microcystic adnexal carcinoma: report of rare cases.

ABSTRACT: Microcystic adnexal carcinoma (MAC) is a rare, locally aggressive malignant neoplasm that derives from cutaneous eccrine/apocrine glands. MAC is classified as an eccrine/apocrine gland tumor and usually occurs in the skin. Here, we characterized and compared two cases of MAC. One is extremely rare in terms of its occurrence in the tongue. The other occurred in the lip, which is common. Histories of disease, diagnosis, and differentials were reviewed by the attending physicians. Hematoxylin and Eosin (HE) slides were evaluated by an experienced pathologist. Immunological markers for malignant eccrine/apocrine gland tumors were used to characterize the tumor's nature. The examined markers included EMA, CK5/6, CK8/18, CK7, CK20, p63, S-100, Calponin, CD10, MYB, Bcl-2, Her-2, CD34, SMA, p53, CD43, CD117, and Ki-67. Both patients were males, presented with painless lumps in the lower lip and in the tongue, respectively. Both lumps were similar in terms of appearance, being whitish, and infiltrative with irregular borders. Both tumors also had similar histological features with nests of bland keratinocytes, cords, and ductal differentiation filled with Periodic acid-Schiff (PAS)-positive eosinophilic material. In both cases, circular or ovary tumor cells invaded into muscles and nerves. All tumor cells were CK5/6, CK8/18, EMA, and CK7 positive. Particularly, keratinocytes were p63 positive, and paraductal cells were p63, S-100, and SMA positive. Therefore, the rare case of MAC in the tongue appears to derive from the salivary gland.

SUBMITTER: Zhang L

PROVIDER: S-EPMC6974419 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature

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Microcystic adnexal carcinoma: report of rare cases.

Zhang Lei L Huang Xiaofeng X Zhou Ting T Cao Hongbao H

Bioscience reports 20200101 1

Microcystic adnexal carcinoma (MAC) is a rare, locally aggressive malignant neoplasm that derives from cutaneous eccrine/apocrine glands. MAC is classified as an eccrine/apocrine gland tumor and usually occurs in the skin. Here, we characterized and compared two cases of MAC. One is extremely rare in terms of its occurrence in the tongue. The other occurred in the lip, which is common. Histories of disease, diagnosis, and differentials were reviewed by the attending physicians. Hematoxylin and E ...[more]

PMID: 31912868

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Project description:BackgroundPrimary hepatic neuroendocrine carcinoma (PHNEC) is extremely rare. The diagnosis of PHNEC remains challenging-partly due to its rarity, and partly due to its lack of unique clinical features. Available treatment options for PHNEC include surgical resection of the liver tumor(s), radiotherapy, liver transplant, transcatheter arterial chemoembolization (TACE), and administration of somatostatin analogues.Case presentationWe report two male PHNEC cases and discuss the diagnosis and treatment options. Both cases presented with abdominal pain; case two also presented with symptoms of jaundice. The initial diagnosis for both cases was poorly differentiated grade 3 small-cell neuroendocrine carcinoma, based on imaging characteristics and the pathology of liver biopsies. Final diagnoses of PHNEC were arrived at by ruling out non-hepatic origins. Case one presented with a large tumor in the right liver lobe, and the patient was treated with TACE. Case two presented with tumors in both liver lobes, invasions into the left branch of hepatic portal vein, and metastasis in the hepatic hilar lymph node. This patient was ineligible for TACE and was allergic to the somatostatin analogue octreotide. This limited treatment options to supportive therapies such as albumin supplementation for liver protection. Patient one and two died at 61 and 109 days, respectively, following initial hospital admission.ConclusionsWe diagnosed both cases with poorly differentiated grade 3 small-cell PHNEC through imaging characteristics, immunohistochemical staining of liver biopsies, and examinations to eliminate non-hepatic origins. Neither TACE nor liver protection appeared to significantly extend survival time of the two patients, suggesting these treatments may be inadequate to improve survival of patients with poorly differentiated grade 3 small-cell PHNEC. The prognosis of poorly differentiated grade 3 small-cell PHNEC is poor due to limited and ineffective treatment options.

Dataset Information

Microcystic adnexal carcinoma: report of rare cases.

Publications

Microcystic adnexal carcinoma: report of rare cases.

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