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CORRESPONDENCE
Year : 2019  |  Volume : 64  |  Issue : 1  |  Page : 80-83
Primary mucinous adenocarcinoma of skin in axilla: A case report and review of literature


Department of Pathology, ESIPGIMSR, MIDC, Andheri (East), Maharashtra, India

Date of Web Publication7-Jan-2019

Correspondence Address:
Dr. Swati Bipin Ghanghurde
Department of Pathology, ESIPGIMSR, MIDC, Andheri (East), Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_320_17

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How to cite this article:
Ambawade VD, Ghanghurde SB, Kate MS. Primary mucinous adenocarcinoma of skin in axilla: A case report and review of literature. Indian J Dermatol 2019;64:80-3

How to cite this URL:
Ambawade VD, Ghanghurde SB, Kate MS. Primary mucinous adenocarcinoma of skin in axilla: A case report and review of literature. Indian J Dermatol [serial online] 2019 [cited 2019 Jan 15];64:80-3. Available from: http://www.e-ijd.org/text.asp?2019/64/1/80/249538




We present a case of 65-year-old male presented to the general surgery outpatient department for the evaluation of a painless, superficial nodular mass in the left axilla that had slowly grown over a period of 2 years and measured 8 cm × 6 cm [Figure 1]. The mass was subcutaneous, attached to the skin, and mobile with respect to the underlying tissue. There was no history of pain, secretion, or overlying redness. On examination, no regional lymphadenopathy, no palpable lump in both the breasts, or abnormal findings in the head or neck were seen. The other axilla was unremarkable. The clinical diagnoses varied from metastasis from an unknown primary to the axillary lymph node, epidermal cyst, and hemangioma. Fine-needle aspiration cytology (FNAC) was done. The smears were hypercellular, tumor cells forming cohesive clusters, isolated scattered singly, and at places forming a vague acinar pattern against the mucoid background [Figure 2]. The cells were monomorphic and lacked atypia. Diagnosis of adnexal tumor was made on cytology. Excision biopsy was done, and the specimen was sent for histopathology examination. Gross examination revealed an ill-defined, soft-tissue mass, partially skin covered, measuring 1 cm × 1 cm in diameter. Cut surface of the tumor had a gelatinous appearance. Microscopic examination revealed a well-circumscribed tumor present in the lower dermis composed of multiple nodules separated by ill-defined fibrocollagenous septae. Nodules were composed of small clusters, islands of tumor cells with cribriform pattern floating in pools of mucin [Figure 3]. Tumor cells were large with abundant eosinophilic cytoplasm and centrally located uniform dark nuclei. Subtle nuclear atypia was noted. Intracytoplasmic mucin was also seen frequently. Focal apocrine differentiation was also noted [Figure 4]. No mitosis or areas of necrosis were seen. The neoplastic cells were strongly positive for cytokeratin 7 (CK7) [Figure 5] and negative for CK20. The mucin was periodic acid–Schiff positive and diastase resistant [Figure 6] and alcian blue positive at pH of 2.5 [Figure 7] and negative at pH of 1. Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography scans of the chest, abdomen, and pelvis, were found to be normal. Thus, the lesion in the skin was reported to be primary mucinous carcinoma of the skin (PMCS).
Figure 1: Clinical photograph of the left axilla with a nodular mass

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Figure 2: Microphotograph showing tumor cells floating in pools of mucin (H and E, ×200)

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Figure 3: Microphotograph showing solid tubular and cribriform arrangement of the tumor cells floating in the pools of mucin (H and E, ×40)

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Figure 4: Microphotograph showing tumor cells with apocrine differentiation (H and E, ×100)

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Figure 5: Microphotograph showing tumor cells positive for cytokeratin 7 (CK7, ×40)

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Figure 6: Microphotograph showing periodic acid–Schiff positive with diastaseresistant mucin (periodic acid–Schiff with diastase, ×40)

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Figure 7: Microphotograph showing alcian blue positive mucin at pH 2.5 (Alcian blue, ×40)

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At this stage, the patient was referred to a cancer hospital. He had undergone an extensive oncological evaluation, including a positron emission tomography scan, to rule out primary adenocarcinoma in the breast and gastrointestinal tract (GIT) and showed negative metastatic work-up. Hence, the diagnosis of a primary mucinous adenocarcinoma of the axilla was confirmed. He underwent Mohs micrographic surgery until negative margins were obtained. The patient was put on the biannual follow-up program for local recurrence or metastasis.

PMCS is an uncommon subtype of sweat gland tumor, with <150 cases described in the literature till date.[1],[2] It is usually seen in the head and neck region, mostly from the eyelid and periorbital region.[3],[4] Only eight cases have been described in the axilla. FNAC is a very useful preoperative investigation, and the diagnosis can be offered on cytology in the appropriate clinical settings.

PMCS is a rare adnexal neoplasm with sweat gland differentiation.[5] Most of the authors favor its eccrine differentiation over apocrine differentiation based on the immunohistochemistry, electron microscopic, and ultrastructural analysis.[1] It has better prognosis than other malignant skin appendage tumors; its accurate identification is very important for further management.[4] This tumor was first described by Lennox et al., in 1952,[6] and later designated by Mendoza and Helwig, in 1971.[7]

PMCS is slightly more common in men than in women and typically affects people in the age range of 50–70 years.[8] The most common location is the head and neck, especially around the eyelids. Other locations are extremity 2%, vulva 4%, axilla 9%, neck 2%, canthus 2%, groin 1%, ear 1%, scalp 17%, face 14%, and chest or abdomen 7%.[1],[2],[3]

Typically, this tumor presents as a slow-growing, painless, soft, sometimes indurated, reddish or gray-blue, nonulcerating nodules that have been present for several years, with long courses for up to 20 years before presentation.[9] The presentation of this neoplasm in the form of ulcer or cyst has also been described.[9] The tumors usually range in size between 1 and 8 cm, the mean diameter before excision being reported to be around 1.8 cm. However, larger variants have been described in the literature. The nodules are well circumscribed, unencapsulated, and often fixed to the dermis making them unable to be “shelled out.”[9]

It has local recurrence rate of 30% after excision with narrow surgical margin, and it rarely metastasizes.[3],[10] The rate of local metastasis to the regional lymph nodes has been reported as 10%.[10] Local metastasis is low due to its vascular characteristics.[2],[3] Distant metastases have been reported in 2%–7% of affected patients.[9] However, these tumors more frequently invade tissues by direct extension, due to the presence of satellite islands of tumor cells present around the main nodule, and via regional lymph node invasion.[9] Death due to mucinous adenocarcinoma of the skin is exceptional with only <5 cases reported thus far, mostly associated with multiple tumor recurrences and widespread metastatic disease.[9] Our patient had no lymph node metastases.

Mucinous carcinoma rarely originates in the skin; the majority of examples in the skin are actually metastasis to it.[11] Primary tumor that may metastasize to the skin and produce mucin is in the breast, gastrointestinal tract (GIT), lung, kidney, ovary, pancreas, prostate, salivary gland, lacrimal gland, urinary tract, paranasal sinuses, or bronchi.[4],[12]

Primary lesions can be differentiated from metastatic lesions by their more organized epithelial cells, less hyperchromasia, and fewer mitoses in individual cells.[9] Features supporting metastasis are abundance of mucin, larger tumor clusters and sheets with predominance of the malignant epithelial cells over mucin, and lack of honeycombing due to the absence of fibrous septae in between the mucinous lakes.[9] Primary staging in an affected individual is essential.[13]

Electron microscopy reveals two types of cells – light and dark cells as found in the eccrine coils.[3] The sialomucin is secreted by the dark cells.[3] In the absence of ancillary techniques, one can diagnose this tumor on typical morphology, by demonstrating sialomucin secretion on special stains and thorough clinical examination.[3]

The treatment of this indolent tumor is wide local excision and regular follow-up.[3] As local recurrences are more common, 1 cm margin is recommended.[3] Mohs micrographic surgery can be particularly advantageous treatment modality in this setting.[9] These tumors are generally resistant to radiotherapy and chemotherapy, and these tools are not usually employed.[9] Finally, patients are to be counseled about the importance of frequent follow-up to rule out local tumor recurrence or the development of regional lymphadenopathy.[9]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

We gratefully acknowledge the kind support of the surgery department of our institute.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Martinez S, Young S. Primary mucinous carcinoma of the skin: A review. Internet J Oncol 2004;2:1-7.  Back to cited text no. 1
    
2.
Maerki J, Ahmed S, Lee E. Primary mucinous carcinoma of the skin. Eplasty 2013;13:ic47.  Back to cited text no. 2
    
3.
Bari V, Poshirkar A. Cutaneous primary mucinous adenocarcinoma of axilla – A rare tumour at rare site. Int J Curr Med Appl Sci 2015;9:7-9.  Back to cited text no. 3
    
4.
Kotru M, Manucha V, Singh UR. Cytologic and histologic features of primary mucinous adenocarcinoma of skin in the axilla: A case report. Acta Cytol 2007;51:571-4.  Back to cited text no. 4
    
5.
Ku BS, Kwon OE, Kim DC, Song KH, Lee CW, Kim KH. A case of primary mucinous carcinoma of the skin. Korean J Dermatol 2005;43:1228-32.  Back to cited text no. 5
    
6.
Lennox B, Pearse AG, Richards HG. Mucin-secreting tumours of the skin with special reference to the so-called mixed-salivary tumour of the skin and its relation to hidradenoma. J Pathol Bacteriol 1952;64:865-80.  Back to cited text no. 6
    
7.
Mendoza S, Helwig EB. Mucinous (adenocystic) carcinoma of the skin. Arch Dermatol 1971;103:68-78.  Back to cited text no. 7
    
8.
Mardi K, Diwana VK. Primary cutaneous mucinous carcinoma: A rare entity. Indian Dermatol Online J 2011;2:82-4.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Beteddini OS, Sheikh S, Shareefi F, Shahab R. Primary mucinous adenocarcinoma of the scalp: A case report and literature review. Int J Surg Case Rep 2015;10:241-4.  Back to cited text no. 9
    
10.
Akinci M, Salan A, Cetin B, Aslan S. Primary mucinous eccrine adenocarcinoma of the skin in a 69-year-old man. Chirurgia (Bucur) 2010;105:109-11.  Back to cited text no. 10
    
11.
Carson HJ, Gattuso P, Raslan WF, Reddy V. Mucinous carcinoma of the eyelid. An immunohistochemical study. Am J Dermatopathol 1995;17:494-8.  Back to cited text no. 11
    
12.
Sarkar M, Nanda D, Parekh D, Das M, Manna A, Sarkar D. Primary mucinous eccrine adenocarcinoma of skin: Study of two cases. Scholars J Appl Med Sci 2016;4:2405-7.  Back to cited text no. 12
    
13.
Scholz IM, Hartschuh W. Primary mucinous eccrine carcinoma of the skin – A rare clinical tumor with many differential diagnoses. J Dtsch Dermatol Ges 2010;8:446-8.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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