Periocular apocrine adenocarcinoma presenting as an orbital mass: clinicopathological features and management in four patients

Purpose: Apocrine adenocarcinoma is a cutaneous adnexal malignancy which can rarely arise from ocular structures. In this retrospective study, we report our experience with four patients who had primary periocular apocrine adenocarcinoma initially presenting with an orbital tumor. Methods: Data extracted included demographics, clinical, imaging, and histopathological features, and treatment outcomes. Results: The definitive diagnosis was established after an incisional biopsy in all cases. Two patients were then managed with exenteration. The third patient underwent local resection followed by radiotherapy but had to be exenterated because of new tumor formation 7 years later. The fourth patient had to be managed with oral bicalutamide which kept the tumor stable for 3 years. Recurrence-free survival for the radical surgical treatment group was 10, 6, and 7 years respectively. Conclusion: Periocular apocrine adenocarcinoma may insidiously develop as an orbital mass without any clinically detectable primary eyelid skin or conjunctival lesions. This tumor must be in the differential diagnosis of medially located ill-defined orbital masses in patients over 50 years of age. Orbital exenteration appeared as an effective treatment of apocrine adenocarcinoma with orbital extension. Anti-androgenic treatment in an androgen receptor-positive tumor provided temporary local tumor control.

A Case of Primary Apocrine Adenocarcinoma Presenting With Axillary Mass

Introduction: Primary apocrine sweat gland adenocarcinoma is a very rare tumour. Apocrin carcinoma is a high incicence of local recurrence and lymph node metastasis. When the location of the tumor is axilla, it should be differentiated from occult breast cancer. Surgery is the first step in primary apocrine cancer treatment. However, there is no clear consensus about adjuvant part of treatment.Methods: The case with axillary apocrin carcinoma was presentated diagnosis, differential diagnosis and treatment approach.Result : Axillary localized apocrine carcinoma was differentiated from occult breast cancer by pathological findings. For this reason, the patient was operated only an axillary dissection operation. Operated patient with axillary apocrine carcinoma was treated with radiotherapy. As a result of pathological evaluation of the tumor, tamoxifen was added to the treatment when the hormone receptor was positive.Conclusions: The patient with apocrine carcinoma was treated with sequential radiotherapy and tamoxifen, and disease-free follow-up to this day

Primary Extra Mammary Paget’s Disease of Vulva, With Apocrine Adenocarcinoma, Signet Ring Cell Differentiation and Distant Metastasis

Objective: Extramammary Paget's disease (EMPD) with invasive carcinoma and distant metastasis is extremely rare. In vulva EMPD associated apocrine carcinoma with signet ring cell differentiation has not been described in the literature so far. Its slow evolution, varied clinical presentation and histological appearances, lead to difficulty in diagnosis of this disease. Case report: We hereby report a case of primary EMPD with invasive carcinoma and distant metastasis in a 59-year-old female who presented with erythematous indurated plaque over vulva. Histopathology revealed Paget cell infiltration throughout the epidermis with invasive carcinoma in dermis and liver metastasis on CECT. The immunohistochemical expressions of CK7, CK20, GCDFP-15, CEA, p40, CDX 2, Her-2/ neu, AR, ER, were examined to explicate the cellular differentiation of this carcinoma. According to the histological assessment, this case was diagnosed as primary EMPD with apocrine adenocarcinoma, signet ring cell differentiation, vulva. Conclusion: Owing to poor prognosis, a high index of clinical suspicion along with histological and immunohistochemical assessment is of utmost importance in arriving at final diagnosis.

A rare case of perianal apocrine adenocarcinoma

Apocrine adenocarcinoma is a rare primary cutaneous malignancy that arises from areas with high apocrine gland density, most frequently described in the axilla. There have only been three previously reported cases of apocrine adenocarcinoma in the anal/perianal region. A 72-year-old female presented for evaluation of a perianal lesion with persistent drainage that she had noticed for over a year. The patient proceeded with surgical excision of the perianal nodule. Diagnosis was made based on pathology demonstrating areas of mixed solid and trabecular areas with large nuclei and many prominent mitotic figures, which stained positive for periodic acid–Schiff–diastase, cytokeratin 7 and gross cystic disease fluid protein 15. We are reporting just the fourth such case of apocrine adenocarcinoma in the anal/perianal region. It is important to consider apocrine adenocarcinoma in our differential, because though apocrine adenocarcinoma has a benign clinical presentation, it can have a high incidence of lymph invasion on presentation.