Primary Cutaneous Apocrine Research Articles

A rare case of primary cutaneous apocrine carcinoma of an unusual site

A rare case of primary cutaneous apocrine carcinoma of an unusual site

Cover Image

The cover image is based on the Case Study Primary cutaneous apocrine carcinoma with RARA::NPEPPS fusion by Volha Lenskaya et al., https://doi.org/10.1111/cup.14607. image

Primary Cutaneous Apocrine Carcinoma: Diagnostic and Management Difficulties of an Uncommon Neoplasm

Primary cutaneous apocrine carcinoma (PCAC) is a rare adnexal neoplasm. Due to its clinical presentation, it can mimic other tumors, especially metastasis. We present a case of primary apocrine carcinoma of the axilla mimicking breast carcinoma metastasis in a 54-year-old woman. The patient presented with a painless, lobed, and erythematous nodule in the left axilla of one year duration. We suspected an adnexal neoplasm but also considered metastasis from breast carcinoma. Histological examination revealed apocrine proliferation. Immunohistochemistry stains of tumor cells were positive for AE1/AE3, and negative for CK7, CK20, p63 and GATA3. Radiological explorations did not reveal any signs of malignancy. Considering all these features, the conclusive diagnosis was primary cutaneous apocrine carcinoma. Treatment was wide excision without adjuvant therapy, and no recurrence was noted. Due to the rarity of this carcinoma, its various clinical presentations, and histological overlaps with breast cancer metastasis, making the diagnosis may be challenging. A comprehensive evaluation of all findings is necessary to rule out other tumors. There is any consensus on the management strategy. The standard treatment is surgical excision, but adjuvant therapies such as radiotherapy or chemotherapy can be proposed in advanced stage disease.

Primary Cutaneous Apocrine Adenocarcinoma of the Axilla in Klinefelter’s Syndrome: A Case Report

Background: Primary Cutaneous Apocrine Adenocarcinoma (PCAA) is a rare cutaneous malignancy that arises from areas rich in apocrine glands, particularly the axilla. There are less than 200 cases described in the literature. However, none has been reported in patients with Klinefelter syndrome, who are known to have an increased risk of breast cancer, Case Presentation: We present a 66-year-old man with a significant family history of breast cancer who developed a right axillary mass initially thought to be hidradenitis suppurativa. On physical examination, he had bilateral gynecomastia. Laboratory workup was significant for hyper-gonadotrophic hypogonadism. Mammography showed bilateral gynecomastia with no radiologic evidence of malignancy in the breast, while magnetic resonance imaging of the breast revealed two suspicious masses seen in the right breast. Excision biopsy of the right axillary mass revealed a high-grade invasive adenocarcinoma involving the dermis and subcutis; the cells had prominent nucleoli vesicular chromatin pattern and voluminous eosinophilic cytoplasm consistent with primary cutaneous apocrine adenocarcinoma. Immunohistochemistry was positive for GATA-3, GCDFP-15, E-cadherin, ER Positive, PR negative, HER 2 by IHC 3 +, and androgen receptor-positive 100 %. Positron emission tomography showed mildly hypermetabolic asymmetric gynecomastia, right greater than left, but no abnormal hypermetabolic activity to suggest malignancy. Karyotype confirmed 47 XXY chromosomes which on. The patient underwent bilateral mastectomy due to his preference; excised breast tissue was negative for malignancy. Conclusion: We presented the first case report of PCAA of axillary in Klinefelter syndrome. Awareness of this association and differentiation from breast carcinoma should be undertaken.

Primary Cutaneous Apocrine Carcinoma of Scrotum with Pagetoid Phenomenon

Primary cutaneous apocrine carcinoma (PCAC) of the scrotum is an unwonted neoplasm that predominantly occurs in areas with high-density apocrine glands. However, the incidence of scrotal PCAC is uncommon. Further, the pagetoid phenomenon is a rare feature of PCAC involving malignant cells infiltrating the epidermis. We present a rare case of scrotal PCAC in an elderly patient who presented with a slow-growing scrotal lesion. A physical examination identified an ulcerated globular mass at the right scrotum with ipsilateral inguinal lymphadenopathy, and a biopsy confirmed the diagnosis of apocrine carcinoma. In addition, imaging studies revealed features of lymph nodes and bone metastasis. Subsequently, a wide local excision was performed. The clinical features of PCAC are not distinctive; thus, histopathology and immunohistochemistry are paramount to confirm the diagnosis. The ideal treatment to achieve clear margins is tumor excision, while other treatment modalities such as chemotherapy, targeted antibody therapy, and radiotherapy are not established.

Primary Cutaneous Apocrine Carcinoma of the Neck Involving the Parotid Gland: A Case Report and Review of the Literature

Primary Cutaneous Apocrine Carcinoma of the Neck Involving the Parotid Gland: A Case Report and Review of the Literature

Poorly differentiated cutaneous apocrine carcinomas: histopathological clues and immunohistochemical analysis for the diagnosis of this unusual neoplasm.

Primary cutaneous apocrine carcinoma (PCAC) is a rare cutaneous malignancy that is derived from apocrine glands. Histologically, these tumours can appear well-differentiated where diagnosis should be relatively straightforward. However, occasionally these tumours can exhibit high-grade features, and in such instances the diagnosis can be challenging. A retrospective analysis of 12 cases of poorly differentiated PCAC, obtained from large academic institutions, was performed, and summarised below. Immunohistochemical studies were performed in all cases with antibodies against CK7, p63, CAM 5.2, GCDFP-15, GATA3, CEA, PR, ER, HER2, calponin, SMA, androgen receptor and EMA. All 12 cases were poorly differentiated; however, there were some histopathological clues to the diagnosis of apocrine carcinoma; namely, the presence of focal glandular formation, acrosyringial involvement and the presence of single 'pagetoid' cells within epidermis. All tumours were consistently positive for CK7, GATA3 and GCDFP-15 and negative for p63. The tumours had variable expression of CAM5.2, CEA, ER, PR, HER2, androgen receptor and EMA. In three cases, there was a preservation of the myoepithelial cell layer (with calponin and SMA), which also confirmed the primary cutaneous origin. PCAC is a difficult neoplasm to diagnose, as it can appear identical to metastatic carcinomas. We describe 12 cases of poorly differentiated PCAC, highlighting their salient clinical, histopathological and immunohistochemical features, and discuss the potential diagnostic pitfalls in distinguishing this entity from other malignant neoplasms. Our results indicate that a combination of thorough histological inspection coupled with an adequate battery of immunohistochemical stains is necessary to support the diagnosis of PCAC.

PSY6-4 Diagnosis and treatment for rare cancers of unknown primary

Carcinoma of unknown primary (CUP) has historically been reported to account for 3-5% of all cancers, more recently for 1-2%, and is itself a rare cancer. The most common subtype of CUP is adenocarcinoma, followed by poorly differentiated carcinoma, squamous cell carcinoma, neuroendocrine tumor. The increasing use of immunohistochemistry (IHC) and chromosome/genetic testing has enabled us to identify rarer diseases of CUP. When an apocrine carcinoma of unknown primary is encountered, the differential diagnosis between carcinoma metastasizing to the skin and primary cutaneous apocrine carcinoma is challenging, but can be distinguished by a clinical-pathological correlation.

A Rare Case of Primary Cutaneous Apocrine Carcinoma

Primary cutaneous apocrine carcinoma is a rare malignant tumor that originates from apocrine sweat glands. Apocrine sweat gland tumor is infrequent: the first case was published by Horn in 1944 and less than 50 cases are described in literature worldwide. For this reason, there are no clear guidelines for the management and treatment of this uncommon cancer. We report a clinical case of primary cutaneous apocrine carcinoma in a 86 year-old female patient.

A clinical, pathological and immunohistochemical series of 9 cases of primary cutaneous apocrine carcinomas of the head and neck.

Primary cutaneous apocrine carcinoma is a rare malignant adnexal skin tumour that can recur locally, spread to regional lymph nodes and metastatize to visceral organs. Wide dissemination and death from disease are much less common. The axilla is the most common site of presentation. It is infrequently reported in the head and neck region. All cases diagnosed as primary cutaneous apocrine carcinoma of the head and neck were retrospectively collected from the archives of the Division of Pathological Anatomy, University of Florence from 1996 to 2016. There was no history or clinical evidence of breast cancer. Clinical data and follow-up were collected by the clinicians. Nine cases were found, with a mean age of 76years, ranging in size between 0.3 and 3.5cm. Clinically, they were frequently mistaken for basal cell carcinomas. Histopathologically, all the tumours showed decapitation secretion, a tubular, solid or mixed (tubulo-papillary and solid-tubular) growth pattern and were predominantly classified as grade 2 tumours. GCDFP-15 and hormone receptors were variably expressed. HER2 and podoplanin were negative in all cases. In one case, spreading to regional lymph nodes was observed. No cases were associated with death due to the disease. As immunohistochemical analysis lacks specificity in distinguishing primary cutaneous apocrine carcinoma from a cutaneous metastasis of breast carcinoma, detailed clinical history, breast examination, adequate treatment and follow-up are necessary to confirm a diagnosis of primary cutaneous apocrine carcinoma.

GATA3 staining in primary cutaneous apocrine cribriform carcinoma: Usefulness to differentiate it from breast cancer metastasis.

Primary cutaneous apocrine cribriform carcinoma (PCACC) is a rare tumor, clinically appearing as a solitary nodule, mostly involving extremities of females and this lesion usually raises a differential diagnosis with metastatic cribriform carcinomas, especially breast cancer. To study GATA3 expression in a series of 14 primary cutaneous cribriform carcinomas and to test its usefulness to differentiate this tumor from metastatic breast cancer. We retrieved 14 cases with PCACC (each from a different patient) from the files of the authors. Cases were dated from 1994 to 2014. We also evaluated 6 cases of cutaneous breast cancer metastasis RESULTS: No PCACCs expressed GATA3. Breast cancer metastases expressed GATA3 in 100% of our studied cases. Even though GATA3 expression has been reported in many benign and malignant adnexal tumors (mostly of sebaceous, follicular, and apocrine differentiation), as well as in many other neoplasms, GATA3 staining to differentiate PCACC from skin breast cancer metastasis has a high negative predictive value. A positive GATA3 staining in this context should permit one to rule out PCACC with a high level of confidence.

Primary Cutaneous Apocrine Carcinoma with Distant Metastasis

Primary Cutaneous Apocrine Carcinoma with Distant Metastasis

Primary cutaneous apocrine carcinoma treated with radiotherapy

Primary cutaneous apocrine carcinoma treated with radiotherapy

有茎性を呈した腋窩原発アポクリン腺癌の1例

有茎性を呈した腋窩原発アポクリン腺癌の1例

Primary Cutaneous Apocrine Carcinoma.

Primary Cutaneous Apocrine Carcinoma.

Treatment of primary cutaneous apocrine carcinoma of the scalp - case and review of the literature

Treatment of primary cutaneous apocrine carcinoma of the scalp - case and review of the literature

左腋窩に生じたアポクリン腺癌の1例

左腋窩に生じたアポクリン腺癌の1例

副乳癌との鑑別に苦慮した左腋窩アポクリン腺癌の1例

75歳，女性。2007年12月左腋窩に皮下硬結が出現，徐々に増大し，当科を受診した。2012年2月29日当科初診時，左腋窩に3×2mmの淡褐色有茎性腫瘤，直下に12×7mmの皮下硬結を認めた。2012年3月6日に全摘生検し，組織学的に真皮から皮下に管腔構造・断頭分泌を伴う好酸性の腫瘍細胞が胞巣を形成して増殖し，深部では膠原線維束間に小胞巣の浸潤を認めた。正常の乳腺や乳管組織はみられなかった。免疫組織化学で腫瘍細胞はEMA，CK7，GCDFP－15陽性，CEA，S－100，ER，PR，HER－2陰性であった。上記所見より本症例をアポクリン腺癌と診断，4月6日に拡大切除および左腋窩リンパ節郭清を施行した。術後15ヵ月まで再発や転移はみられていない。

頭部に生じた皮膚原発アポクリン腺癌の1例

頭部に生じた皮膚原発アポクリン腺癌の1例

Successful Treatment of HER-2-Positive Metastatic Apocrine Carcinoma of the Skin with Lapatinib and Capecitabine

© 2012 The Authors. doi: 10.2340/00015555-1354 Journal Compilation © 2012 Acta Dermato-Venereologica. ISSN 0001-5555 Primary cutaneous apocrine carcinoma (AC) is a rare and highly aggressive cutaneous adenocarcinoma. In general, most cutaneous AC occurs in the axilla and it is more common in middle-age women (1). Complete remission by chemotherapy is uncommon and median survival was only 2 years for patients with Bloom-Richardson grade 3 tumours (2). HER-2 is a 1260-amino-acid transmembrane protein with tyrosine kinase that plays a central role in cell differentiation, adhesion and motility (3). In the treatment of breast cancer, HER-2 signal inhibitors, such as the humanized monoclonal antibody trastuzumab and the small molecule tyrosine kinase inhibitor lapatinib, have been used as a standard therapy for HER-2 overexpressing metastatic tumour (4). Moreover, several reports have indicated that the apocrine subtype of breast cancer consistently overexpressed HER-2 (5). Interestingly, AC has a marked histological similarity to the apocrine subtype of breast cancer, including immunohistochemistry (6). Therefore, we hypothesized that a HER-2 signal inhibitor, such as lapatinib, can be effective for HER-2 overexpressing metastatic AC. We report here a patient with a metastatic AC showing a complete remission following the administration of lapatinib with capecitabine. To our knowledge, this is the first case report describing the successful treatment of metastatic AC with HER-2 signal inhibitors.