| Abstract|| |
Primary cutaneous apocrine carcinomas are rare cutaneous adnexal neoplasm with less than 100 cases reported in the literature. The tumor arises in areas rich in apocrine glands particularly axilla, modified apocrine glands such as ceromucinous and Moll's glands. Most of these carcinomas are indolent and slowly growing but few rapidly progressive and extremely aggressive? cases have also been reported. Wide local excision with clear margins and sentinel lymph node biopsy is standard treatment of choice.This case was of much interest from the diagnostic point of view both microscopically as well as immunohistochemically. We report a case of 46-year-old man who presented to our hospital with an ulcerated painless nodular mass in his right axilla.With combined use of microscpic features, special stains and immunohistochemical examination the case was correctly diagnosed as primary cutaneous apocrine carcinoma.
Keywords: Apocrine carcinoma, GCDFP-15, Sweat gland carcinoma, Axillary mass
|How to cite this article:|
Chadha S, Kumar R, Singhal S, Ruhela S. Primary cutaneous apocrine carcinoma: Case report and literature review. Indian J Pathol Microbiol 2021;64:183-5
|How to cite this URL:|
Chadha S, Kumar R, Singhal S, Ruhela S. Primary cutaneous apocrine carcinoma: Case report and literature review. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Apr 21];64:183-5. Available from: https://www.ijpmonline.org/text.asp?2021/64/1/183/306506
| Introduction|| |
Primary cutaneous apocrine carcinoma, a subtype of sweat gland carcinoma is a rare and incompletely studied neoplasm. The estimated incidence rate ranges from 0.0049-0.0173 per 100,000 patients per year. Both genders are affected equally. Most of these cutaneous adnexal neoplasms arise in the areas rich in apocrine glands.The axilla is most common site affected followed by the anogenital area, scalp, chest, eyelid, and ear. Mostly apocrine carcinomas are indolent and may achieve long term remisssion only with surgical treatment, but rapidly progressive and clinically agressive cases has also been reported. Wide surgical excision with clear margins and sentinel lymph node biopsy is the standard treatment of choice.,,,
| Case Report|| |
A 46-year-old man presented with an ulceroproliferative painless mass in right axilla with occasional serosanguinous discharge. The mass was slowly growing that had been present for 5-6 years. On physical examination, a 7.0 × 6.0 × 4.0 cm, indurated firm ulceroproliferative mass with central nodularity was present in his right ailla. He was previously treated with full course of antibiotic for abscess but with no resolution. PET CT revealed a large, lobulated, heterogenously enhancing exophytic mass lesion measuring 9.0 × 7.0 × 3.0 cm, involving skin and subcutaneous complex of right axilla. MRI findings were suggestive of enhancing soft tissue mass lesion in the right axilla involving the skin and subcutaneous tissue and enlarged right axillary lymph nodes suggestive of mitotic lesion. Physical examination and radiologial work-up revealed no significant lesion in either breast.The patient had no family history of malignancy.The lesion was excised and the specimen was sent to our lab for histopathological examination.
Grossly, total size of the specimen including skin measured 9.2 × 5.9 × 4.0 cm.Cut sections showed a firm grayish white tumor measuring 7.8 × 5.2 × 3.0 cm, involving the dermis and underlying subcutaneous tissue [Figure 1].
|Figure 1: Gross examination of the specimen revealed a greyish white tumor involving dermis and underlying subcutaneous tissue|
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On microscopic examination sections showed the epidermis stretched with focal areas of ulceration. The underlying dermis revealed diffusely infiltrative tumor composed of solid sheets of round, oval to polygonal neoplastic cells with abundant clear to eosinophilic granular cytoplasm, eccentrically placed oval nuclei showing moderate pleomorphism and prominent nucleoli [Figure 2]. Increased number of mitotic figures were also noted (4/10 HPF).
|Figure 2: Diffusely infiltrative tumor composed of solid sheets of round, oval to polygonal neoplastic cells with abundant clear to eosinophilic granular cytoplasm, eccentrically placed oval nuclei showing moderate pleomorphism and prominent nucleoli (H&E 400×)|
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The tumor cells showed PAS-positive, diastase resistant and iron-positive intracytoplasmic granules [Figure 3]a and [Figure 3]b. Immunohistochemical staining study showed that the tumor cells were positive for GCDFP-15 [Figure 4], Cytokeratin and EMA. Negative immunostaining was obtained with CEA, S-100, HMB-45, Melan-A, Vimentin, SMA, Synaptophysin, Chromagranin, CD-34, CD-45 and CD-99. Estrogen (ER) and progesterone (PR) recptors were not detected. Ki-67 index was 5-6%.
|Figure 3: (a) The tumor cells are stained bright pink showing PAS positivity (PAS, 200×). (b) The tumor cells show colloidal iron stain positive intracellular granules (Hale's colloidal iron, 200×)|
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|Figure 4: The tumor cells demonstrate positive immunostaining with GCDFP-15 (IHC, 400×)|
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| Discussion|| |
Primary cutaneous apocrine carcinoma represents an extremely rare cutaneous adnexal neoplasm with less than 100 cases reported in the literature. The first case of PCAC was described by Horn in 1944.
PCAC is a subtype of sweat gland carcinoma arising in the areas of high apocrine gland density particularly axilla but can also occur elsewhere in the skin like anogenital area, scalp, chest, nipple, eyelid and ear. The age of presentation ranges from fifth to seventh decade of life, with a slightly male preponderance. The usual presentation is a single painless slowly gowing nodule or mass in association with a long standing erythematous plaque.The tumor usually develops de novo but can also arise from preexisting benign lesions such as apocrine hyperplasia or apocrine adenoma., Because of their indolent course, most apocrine carcinomas are diagnosed after many years. One third to half of these patients have lymph node metastases at the time of diagnosis.
Primary cutaneous carcinoma is difficult to diagnose clinically and require histopathological evaluation. Histologically, PCAC must be differentiated from metastatic breast carcinoma, lymphoma, melanoma, extramammary paget's disease, and benign apocrine neoplasms such as apocrine adenoma. Few cases with signet ring cell features have also been reported, raising concern for metastatic stomach or colorectal adenocarcinoma. Diffrentiation of these tumors from PCAC requires a detailed history coupled with a thorough clinical examination, imaging studies, histopathological examination, special stains, and immunohistochemistry. Popularly used immunohistochemical markers used in mammary ductal carcinoma like ER, PR, and mammaglobin can be weakly positive in pcac.
According to Paties et al. the most reliable criteria for identifying primary cutneous apocrine carcinoma is decapitation secretion, PAS-positive, diastase-resistant granular material in the cytoplasm of tumor cells and positive immunoreactivity with gross cystic disease fluid protein 15 (GCDFP-15). Wide surgical excision with clear margins and sentinel lymph node biopsy is the standard treatment of choice. Mohs micrographic surgery is used for the treatment of tumors located in cosmetically sensitive areas like eyelid, fingertip and lip. Usually apocrine carcinomas have an indolent course and may achieve long term remission only with surgical treatment, particulary if the tumor is well differentiated. Moderately and poorly differentiated tumors can be associated with local recurrences and lymph node metastasis therefore adjunctive treatment with radiotherapy and chemotherapy is required in these cases.
| Conclusion|| |
Primary cutaneous apocrine carcinoma reprsents a very rare tumor with less than 100 cases reorted in the literature. Usually these tumors have an indolent course but few cases with local recurrence and distant metastasis have also been reported. A definitive diagnosis and differentiation from other tumors are depenent upon the microscopic findings and immunohistochemistry.
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