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Microcystic adnexal carcinoma: in vitro growth characteristics and effect on stromal collagen production.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a locally aggressive cutaneous neoplasm, which is characterized by follicular and sweat gland differentiation and a stroma of dense collagen. METHODS: MAC cells were studied and characterized in vitro through phase microscopy and proliferation assays. Additionally, to delineate a possible role of soluble mediators in bringing about the dense stromal collagen seen in association with these tumors, the effect of conditioned media derived from the tumor cells on the level of collagen production by normal fibroblasts was examined. RESULTS: A significant morphologic difference between the MAC cells and normal keratinocytes was not noted. Proliferation assays demonstrated a significantly slower rate of growth of the MAC cells in comparison to controls. Media conditioned by MAC cells did not induce an increase in collagen synthesis by cultured fibroblasts, but rather, there appeared to be an inhibitory effect on the level of collagen synthesis. CONCLUSIONS: The increased stromal collagen seen in MAC histologically is not the result of growth factors or cytokines produced by the MAC cells.

Carcinoma↗

Microcystic adnexal carcinoma: report of four cases treated with Mohs' micrographic surgical technique.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare and aggressive malignant tumor of the sweat glands. Clinically, it often presents as a firm subcutaneous nodule on the head and neck regions. On histology, MAC exhibits both pilar and sweat duct differentiation with a stroma of dense collagen. It often extends beyond the clinical margins with local spreading in the dermal, subcutaneous, and perineural tissue planes. It has a high local recurrence rate after standard excision. Recent preliminary reports have indicated more favorable cure rates with Mohs' micrographic surgery (MMS). OBJECTIVE: To present our data on four cases of MAC treated by MMS. We also compared our findings with more recently reported series in the English language literature. METHODS: We reviewed the medical records of four patients (two males and two females) with MAC treated by MMS over the last 3 years. We also obtained follow-up data. RESULTS: In all four patients with MAC treated by MMS, there were no recurrences, with a mean follow-up of 1 year. CONCLUSION: We report an additional four MAC cases treated by MMS. The accumulated data continue to confirm that, if the diagnosis of MAC is made early, and if the anatomic location is accessible to excision by MMS, a favorable outcome can be expected.

Adult↗

Histopathologic, immunophenotypic and genotypic analyses in ocular adnexal lymphoproliferative disorders.

This study reports on fourteen biopsies from patients presenting to our orbital and oncology service with ocular adnexal lymphoid proliferations between November 1988 to September 1991. The biopsies were studied using histologic, immunophenotypic and genotypic analyses. By histologic criteria, there were two reactive, five indeterminate and seven lymphomatous lesions. On immunophenotypic analysis, there were two monoclonal and 10 polyclonal lesions in the 12 specimens analysed. Genotypic analysis confirmed the histopathologic diagnoses for the reactive lesions by showing them to be germline. It also confirmed that both the histopathologic lymphomas and immunophenotypically monoclonal lesions were clonally rearranged. Genotypic analysis was able to separate the histologically indeterminate group into two subsets: clonally rearranged, of which there was one, and germline, of which there were four. In addition, it demonstrated that immunophenotypic polyclonality cannot always be equated with genotypic polyclonality as was the situation in four out of 10 lesions in our series. The significance of clonal arrangements in the histologically indeterminate and immunophenotypically polyclonal groups can only be determined by prospective study.

Adult↗

The adnexal mass: benign or malignant? Evaluation of a risk of malignancy index.

OBJECTIVE: To validate a risk of malignancy index incorporating menopausal status, serum CA 125 level and pelvic ultrasound features in the pre-operative diagnosis of ovarian cancer. DESIGN: A retrospective observational study. SETTING: Department of Obstetrics and Gynaecology, The Royal London Hospital. SUBJECTS: One hundred and twenty-four women admitted consecutively to the gynaecology department for surgical exploration of an adnexal mass. MAIN OUTCOME MEASURES: The sensitivities and specificities of menopausal status, serum CA 125 level and ultrasound features, in isolation and combined (the risk of malignancy index (RMI)), for diagnosing ovarian cancer. RESULTS: Tested on a new population of women, the RMI retained the high sensitivity for diagnosing ovarian cancer seen in the original report describing its derivation. The specificity, however, was lower. The study confirmed that the RMI is more accurate than the individual criteria in diagnosing ovarian cancer, and was comparable with other scoring systems. CONCLUSIONS: The risk of malignancy index is a simple scoring system for ovarian cancer. Application of the risk of malignancy index in clinical practice may provide a rational basis for specialist referral of patients with ovarian cancer before diagnostic surgery.

Antigens, Tumor-Associated, Carbohydrate↗

Microcystic adnexal carcinoma: a case showing eccrine duct differentiation.

Microcystic adnexal carcinoma (MAC) is a cutaneous neoplasm comprised of pilar and sweat duct structures. Characteristically, the ductal structures manifest immunoreactivity for carcinoembryonic antigen (CEA) on immunostaining. The case of MAC discussed here shows strictly sweat duct differentiation. Using a monoclonal antibody to salivary mucin (CF-1) that is specific for eccrine duct, we identify the neoplastic structures to be of eccrine sweat duct histogenesis.

Aged↗

beta-2-microglobulin in benign and malignant adnexal skin tumors and metastasizing basocellular carcinomas.

Immunostaining for beta-2-microglobulin (beta-2m) was performed on various benign and malignant adnexal skin tumors and 3 cases of metastasizing basocellular carcinomas. All the tumors and their metastases, with the exception of 2 basocellular carcinomas gave evidence of beta-2m. Most tumors contained both stained and unstained cells. The stained cells showed differentiation, the unstained ones appeared to be undifferentiated and basal-like in character. The staining of cutaneous tumors for beta-2m may offer a tool for investigating tumor differentiation, but seems not to be of much value in distinguishing malignant from benign lesions.

Basal Cell Carcinoma↗

The genodermatopathology of adnexal tumors.

Several adnexal tumors (e.g., trichoepitheliomas, trichilemmomas, steatocystomas, sebaceous adenomas, and cylindromas) tend to occur as multiple lesions showing autosomal dominant inheritance, sometimes in association with other cutaneous or internal findings. Solitary tumors with the same clinical and histopathologic features are usually trivial non-hereditary lesions. Multiple trichilemmomas are part of Cowden's syndrome, which includes a high incidence of breast cancer in women; multiple sebaceous adenomas and other sebaceous tumors associated with carcinomas of the gastrointestinal tract are part of a syndrome that was first delineated by Bakker and Tjon A Joe.

Adenoma↗

Microcystic adnexal carcinoma: a light microscopic, immunohistochemical and ultrastructural study.

We report a case of microcystic adnexal carcinoma (MAC) occurring on the upper lip of an 82-year-old woman. Microscopically the tumor showed both pilar and sweat gland differentiation, involved the entire dermis and subcutaneous tissue, and invaded perineural spaces. Immunoperoxidase studies revealed carcinoembryonic antigen to be present in the ductal lining cells and in the amorphous content in the lumen, confirming sweat gland differentiation. The S-100 protein was positive in dendritic cells within the solid cell nests, but negative in cells lining cystic spaces. Ultrastructural study confirmed that the neoplasm was composed of two components, with pilar and eccrine differentiation. The former showed concentric layers of squamous epithelial cells with well-developed desmosomes and cytofilaments. The latter had ductal and alveolar structures; the ultrastructural features included: i) numerous villous folds of plasma membrane to interdigitate each other by focal desmosomes, ii) aggregates of cytofilaments, and iii) basally located myoepithelial cells which were separated from the surrounding stroma by rather thick basement membrane. In addition, distinct amyloid deposition was also observed on ultrastructural examination. To our knowledge, amyloid deposition has not been previously reported in MAC.

Aged↗

Benign cutaneous adnexal tumors in childhood and young adults, excluding pilomatrixoma: review of 28 cases and literature.

Benign adnexal tumors of the skin, excluding pilomatrixomas, were identified in 28 patients between the ages of 4 and 20 years with a mean age of 15; 23 women and 5 men. Most lesions were described as papules and they occurred on the face, scalp and neck in 75% of cases. Trichoepithelioma and syringoma accounted for 56% of all neoplasms. Only one patient gave a history of familial trichoepitheliomas. The remaining tumors were examples of syringocystadenoma papilliferum, eccrine acrospiroma, and eccrine and apocrine hidrocystomas. Excisional biopsy constituted the treatment in all cases; only one neoplasm, an eccrine acrospiroma, recurred locally.

Adenoma↗

Epithelial markers and differentiation in adnexal neoplasms of the skin: an immunohistochemical study including individual cytokeratins.

Applying immunohistochemical procedures for the detection of eight different cytokeratin (CK) polypeptides and other differentiation markers, we compared the staining patterns of normal cutaneous structures with those of benign adnexal tumors (n = 65). Syringomas exhibited a marker pattern highly reminiscent of that seen in normal dermal eccrine ducts (EMA in peripheral cells, CK 10 in intermediate cells, and CK 6, CK 19, and CEA in luminal cells). Nodular hidradenomas exhibited complex patterns suggesting relationship between tumor cells, including clear cells, and normal secretory coil cells (CK 7, CK 8, CK 19, and EMA); however, dermal-duct and epidermoid differentiation were also detectable. In both cylindromas and spiradenomas, zonal staining patterns were apparent: modified myoepithelial cells were positive for smooth-muscle-type actin, while the luminal cells mainly expressed ductal markers (CK 6 and CK 19) and, less prominently, secretory-coil markers including CK 7. Eccrine poromas exhibited a widespread reaction for CK 5/6 and EMA, analogous to peripheral dermal duct cells, but focal maturation toward inner-ductal and secretory-coil cells was also demonstrable. The staining pattern observed in trichoepitheliomas resembled that of the outer but not the inner root sheath. In conclusion, the detailed marker profiles obtained in the present study have broadened our understanding of the differentiation and nature of these highly singular tumor types.

Adenoma↗

Mucinous differentiation in adnexal sweat gland tumors.

We report an eccrine acrospiroma, on the cheek of a 29-year-old female, in which the presence of abundant mucinous (goblet cell) metaplasia closely mimicked a primary mucoepidermoid carcinoma. To determine the frequency of mucinous differentiation in benign adnexal sweat gland tumors, we evaluated sixty-five cases in hematoxylin and eosin stained sections for the presence of goblet cells and sixty of these for mucicarmine positivity. Goblet cell metaplasia was seen in 3 of 12 acrospiromas, 1 of 8 mixed tumors, and in 1 of 9 cases of syringocystadenoma papilliferum. All goblet cells were positive for mucicarmine, except in one case of acrospiroma, where goblet cells were not detected on the section stained with mucicarmine. In addition, intracellular mucin, inclusive of goblet cells, was seen in 5 of 12 acrospiromas, 1 of 11 poromas, 5 of 8 mixed tumors, 3 of 13 spiradenomas, 1 of 5 cylindromas, 3 of 9 cases of syringocystadenoma papilliferum and 1 of 3 nipple adenomas. The majority of the tumors had both extracellular mucicarmine positivity (40 of 60) and luminal mucicarmine positivity (39 of 60). We conclude that mucinous differentiation in sweat gland tumors, as defined by the presence of goblet cells and/or intracellular mucicarmine positivity, is common and does not indicate aggressive behavior. Mucinous differentiation in benign sweat gland tumors should not be confused with more aggressive mucoepidermoid carcinomas of salivary gland origin or adenosquamous carcinoma.

Acrospiroma↗

Messenger RNAs for the multifunctional cytokines interleukin-1 alpha, interleukin-1 beta and tumor necrosis factor-alpha are present in adnexal tissues and in dermis of normal human skin.

Interleukin-1 alpha (IL-1 alpha), interleukin-1 beta (IL-1 beta) and tumor necrosis factor-alpha (TNF-alpha) are 3 cytokines that play a key rôle in cutaneous homeostasis and in the immunopathogenesis of a number of dermatologic diseases. Most studies have focused on their production by keratinocytes and Langerhans cells. To determine whether there are non-epidermal sites of cytokine transcription, biopsy specimens of normal human skin were probed for IL-1 alpha, IL-1 beta and TNF-alpha messenger RNAs using the method of in situ hybridization. The results demonstrate that each cytokine mRNA is present at multiple sites within the skin, including epidermal appendages and adnexal structures (hair follicles, sebaceous glands), the dermal microvasculature, arrectores pilorum smooth muscle, and the dermal connective tissue. These data provide evidence that in vivo there are multiple sites other than the epidermis of constitutive IL-1 alpha, IL-1 beta, and TNF-alpha gene transcription in normal human skin.

Hair Follicle↗

A case of endometrioid carcinoma of the fallopian tube mimicking an adnexal tumor of probable Wolffian origin.

We report a very uncommon case of endometrioid adenocarcinoma of the fallopian tube that mimicked, based on histology, a female adnexal tumor of probable Wolffian origin (FATPWO). We present our microscopic and immunohistochemical findings, and a review of the literature concerning these two entities. The differential diagnosis can be of great consequence, owing to the very different prognoses of the two tumors, and is based mainly on macroscopic appearance and immunohistochemical profile: epithelial membrane antigen (EMA) and CA125, generally lacking in FATPWO, are expressed in endometrioid adenocarcinoma, thus indicating the müllerian origin of this tumor.

Aged↗

Female adnexal tumour of probable Wolffian origin.

Two further cases of 'female adnexal tumour of probable Wolffian origin' are described Both were retroperitoneal and presented a unique histology of uniformly close-packed bland spaces. solid islands, cords, and diffuse areas. A small hamartoma in a female fetus, part of which resembled the tumours, was traced to an area of near apposition with some paroophoron canals, providing further evidence of a Wolffian origin. The literature of this and some other putative Wolffian tumours is briefly reviewed, and the relation to rete adenomas is discussed.

Adnexa Uteri↗

Polyomavirus infection in hamsters and trichoepitheliomas/cutaneous adnexal tumours.

Multiple skin nodules, with histological features of adnexal tumours consistent with trichoepithelioma, were observed on the head and trunk of Syrian hamsters. Skin biopsies from 20 hamsters from five different colonies were affected, and two of the affected hamsters also had lymphoma. Two owners reported that 16 of 70 hamsters and 50 of 100 hamsters in their colonies had similar skin lesions. These tumours have previously been associated in laboratory colonies with hamster polyomavirus (HaPV) infection. Examination of skin tissues by electron microscopy failed to reveal intranuclear virus particles. Using recombinant major capsid protein VP1 of HaPV, VP1-specific antibodies were detected in sera from 12 of 12 affected hamsters and in four of four unaffected in-contact hamsters, by ELISA. The ELISA data were verified by immunoblot analysis. Eleven of 13 serum samples contained antibodies which reacted with at least one recombinant structural HaPV protein (VP2), including samples from three in-contact unaffected hamsters. Nine of the 11 anti-VP2-positive samples also reacted with recombinant VP3 of HaPV, and six reacted with VP1. Amplification by PCR and sequencing detected VP1 -encoding sequences showing a high degree of homology with HaPV. The findings suggest a possible infection by HaPV or a HaPV-like virus and it is likely that such an infection was enzootic within the affected colonies.

Animals↗

Primary versus secondary ovarian malignancy: imaging findings of adnexal masses in the Radiology Diagnostic Oncology Group Study.

PURPOSE: To analyze ultrasonographic (US), computed tomographic (CT), and magnetic resonance (MR) imaging features of primary and secondary ovarian malignant neoplasms to determine if there is any significant difference in their appearance. MATERIALS AND METHODS: Analysis of the multi-institutional Radiology Diagnostic Oncology Group data revealed 86 patients with primary ovarian carcinoma and 24 patients with a secondary ovarian neoplasm. Numerous imaging features that had been recorded for the adnexal masses with each imaging modality were reviewed and compared between primary and secondary malignant ovarian neoplasms. RESULTS: Of the imaging features assessed with all three modalities, multilocularity as determined at US (P =.02) or MR imaging (P: =.01) was the only significant feature. At US, 30 (37%) of 81 primary ovarian cancers were multilocular, whereas only three (12%) of 24 metastatic neoplasms were multilocular. At MR imaging, 40 (74%) of 54 primary ovarian cancers were multilocular, whereas only five (36%) of 14 metastatic neoplasms were multilocular. Neither a predominately solid appearance nor bilaterality was significantly different between primary and secondary neoplasms. CONCLUSION: For malignant ovarian masses, multilocularity at MR imaging or US favors the diagnosis of primary ovarian malignancy rather than secondary neoplasm, but it is difficult to accurately distinguish between primary and secondary ovarian malignancies.

Adult↗

Microcystic adnexal carcinoma: review of 51 Japanese patients.

BACKGROUND: Microcystic adnexal carcinoma (MAC) has been reported to occur predominantly in Caucasians. However, the number of cases in Japanese subjects seems to have recently been increasing. OBJECTIVE: To review the Japanese cases of MAC. METHODS: We collected and analyzed the data of 51 Japanese patients recorded in case reports or abstracts over a number of years. RESULTS: Twenty-five of the cases were reported between 1987 and 1996, and the remaining 26 cases between 1997 and 2000. Forty-three lesions were located on the face, 2 on the scalp, 2 on the chest, 1 on the axilla, 1 on the buttock, 1 on the palm and 1 on the toe. One of the above occurred at the site of previous radiotherapy. Fifteen tumors were initially misdiagnosed histopathologically. CONCLUSION: Our review shows that MAC also occurs in the Japanese, in whom it may be more common than previously observed.

Adult↗

Microcystic adnexal carcinoma with lymph node metastasis.

Microcystic adnexal carcinoma (MAC) affects predominantly the face and seldom metastasizes. We report a case occurring in the axilla of a 63-year-old male. Histology revealed the characteristic features of MAC. Eleven months after the excision, he underwent a reexcision with wide margins because of local recurrence. Histologically, the central area of the recurrent lesion revealed the typical histologic features of MAC, and the periphery showed a proliferation of irregular duct-like and glandular structures with a mixed pattern. Two lymph nodes that were not adherent to the tumor had metastatic tumor cells. The present case confirms that MAC can metastasize, although it may also be hypothesized that the recurrent lesion represented transformation into a higher-grade carcinoma.

Carcinoma↗