|Year : 2020 | Volume
| Issue : 2 | Page : 78-80
Primary cutaneous mucinous carcinoma: A rare case report
KN Roopa1, Meena N Jadhav1, Shreekant K Kittur1, Gajanan A Pise2
1 Department of Pathology, Belgaum Institute of Medical Sciences, Belagavi, Karnataka, India
2 Department of Dermatology, Belgaum Institute of Medical Sciences, Belagavi, Karnataka, India
|Date of Submission||16-Jan-2020|
|Date of Decision||19-Mar-2020|
|Date of Acceptance||17-Apr-2020|
|Date of Web Publication||22-Dec-2020|
Dr. Meena N Jadhav
S-04, Sadashiv Residency, Near Shivalaya Temple Road, Sadashiv Nagar, Belagavi - 590 001, Karnataka
Source of Support: None, Conflict of Interest: None
Primary cutaneous mucinous carcinoma is a rare slowly growing neoplasm showing predilection for the head and neck, particularly the eyelids. On occasion, it affects other sites including scalp, face, ear, axillae, thorax, abdomen, groin, foot, hand, and vulva. Fewer than 150 cases have been reported in the literature. A 70-year-old man presented with an asymptomatic raised lesion over the left side of the face for 1 year. Microscopically, the diagnosis of primary cutaneous mucinous carcinoma was made, which was later confirmed by immunohistochemistry. Mucinous carcinoma of the skin has a relatively good prognosis with rare distant metastases, but has risk of local recurrence. Diagnosing it and differentiating it from metastatic carcinoma helps the clinician in further management of the patient.
Keywords: Carcinoma, cutaneous, mucinous, primary
|How to cite this article:|
Roopa K N, Jadhav MN, Kittur SK, Pise GA. Primary cutaneous mucinous carcinoma: A rare case report. Indian J Dermatopathol Diagn Dermatol 2020;7:78-80
|How to cite this URL:|
Roopa K N, Jadhav MN, Kittur SK, Pise GA. Primary cutaneous mucinous carcinoma: A rare case report. Indian J Dermatopathol Diagn Dermatol [serial online] 2020 [cited 2021 Apr 11];7:78-80. Available from: https://www.ijdpdd.com/text.asp?2020/7/2/78/304342
| Introduction|| |
Primary cutaneous mucinous carcinoma was first documented by Lennox et al. in 1952 and was first designated by Mendonza and Helwig in 1971. It is a rare, slowly growing malignant neoplasm showing predilection for head and neck, particularly eyelids. On occasion, it affects other sites including scalp, face, ear, axillae, thorax, abdomen, groin, foot, hand, and vulva. It is usually solitary and growsslowly over several months or even years. Fewer than 150 cases have been reported in the literature. Here, we report a case of primary cutaneous mucinous carcinoma that presented on the left cheek in a 70-year-old male patient.
| Case Report|| |
A 70-year-old man presented with the chief complaint of a non-tender raised lesion over the left cheek for 1 year. There was no history of weight loss, anorexia, fever, abdominal pain, dysphagia and constipation, respiratory or urinary complaints. There was no history of trauma or radiation to the affected area. General physical examination and systemic examination were within normal limits. Local examination revealed a single nodule of size 2.5 × 2.5 cm over the left cheek. Skin over the nodule was normal. There was no regional lymphadenopathy and no nodules found elsewhere. The nodule was excised and sent for histopathological examination. Grossly, a single skin-covered nodule of size 2.5 × 2.5 × 2 cm was received. Microscopic examination of the lesion showed a partly encapsulated tumor in the dermis composed of islands of cells arranged in solid sheets, trabeculae, and cribriform patterns with abundant extracellular pools of mucin separated by thin fibrous septae [Figure 1]. The tumor cells were cuboidal with pink staining, a few with vacuolated cytoplasm and centrally located round to oval vesicular nuclei [Figure 2]a and [Figure 2]b. There was no abnormal mitosis or necrosis seen. Resected margin showed the presence of tumor. The pools of mucin stained positive for Periodic acid–Schiff, resistant to diastase and Alcian blue at pH 2.5 [Figure 3]a and [Figure 3]b. These findings were suggestive of mucinous carcinoma of the skin. The patient was advised computed tomography abdomen, thorax, and gastrointestinal (GI) endoscopy to look for primary mucinous carcinoma in other organs and for evidence of metastasis to other sites. Immunohistochemistry (IHC) was advised to rule out metastatic mucinous carcinoma to skin. The tumor cells were positive for cytokeratin (CK) 7, estrogen receptor (ER), and progesterone receptor (PR) [Figure 4]a, [Figure 4]b, [Figure 4]c and negative for CK20, p63, CK5/6, thyroid transcription factor (TTF)-1, Napsin-A, prostate-specific antigen (PSA), and Caudal-related homeobox (CDX) gene 2. Based on these findings, a final diagnosis of primary cutaneous mucinous carcinoma was made. The clinical workup in our case did not reveal possible primary tumor in other organs or evidence of metastasis to other sites. Follow-up of the patient for 1 year post-surgery showed healing with no local recurrence.
|Figure 1: Microphotograph showing partly encapsulated tumor with pale staining mucin and islands of tumor cells (H and E, ×100)|
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|Figure 2: (a) Microphotograph showing oval to round cells arranged in cribriform pattern with bland nuclei, eosinophilic cytoplasm (H and E, ×400). (b) Microphotograph showing cells with clear cytoplasm arranged in solid pattern (H and E, ×400)|
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|Figure 3: (a) Microphotograph showing mucinous material positive for diastase-resistant Periodic acid–Schiff stain. (b) Microphotograph showing mucinous material positive for Alcian blue stain at pH 2.5 (×100)|
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|Figure 4: (a) Microphotograph showing tumor cells positive for estrogen receptor (ER ×400). (b) Microphotograph showing tumor cells positive for progesterone receptor (PR × 400). (c) Microphotograph showing tumor cells positive for CK7 (CK7 ×400)|
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| Discussion|| |
Primary cutaneous mucinous carcinoma is a tumor seen more in men between the ages of 50 and 70 years, as a slow-growing, painless, nodular, red/gray/purple lesion that may be ulcerated/crusted or with features of telangiectasia. Histologically, the tumor is situated in the dermis and often involves the subcutaneous fat. Lakes of pale-staining mucin with suspended islands of tumor cells compartmentalized by delicate fibrous septae are seen. The mucin is Periodic acid–Schiff-positive diastase resistant, hyaluronidase-resistant, Alcian blue positive (pH 2.5) and also stains with Mucicarmine and colloidal iron. Mucin histochemistry of our case showed mucin positive for Periodic acid–Schiff (resistant to diastase) and Alcian blue at pH 2.5 suggestive of sialomucin in contrast to GI tumors which produce sulfomucin, positive for Alcian blue at pH 1.0.
Primary cutaneous mucinous carcinoma may be histologically indistinguishable from metastatic lesions, particularly of mammary derivation. The tumor cells here showed strong ER and PR positivity which is seen in both breast carcinoma and primary cutaneous mucinous carcinoma. Since the clinical examination and radiological investigations in our patient showed no evidence of a breast lesion, metastasis from breast carcinoma was ruled out. Metastatic mucinous tumors from the GI tract, lungs, salivary glands, and prostate were also considered. In the current case, on light microscopy, there was no dirty necrosis or goblet cell differentiation. IHC showed tumor cells positive for CK7 and negative for CK20, which ruled out metastatic tumor from stomach and colon. CDX2 negativity further confirmed the unlikelihood of colon cancer. Cutaneous metastasis from lung carcinoma was ruled out as the tumor cells were negative for TTF-1 and Napsin-A. Tumor cells were negative for CK5/6, thereby ruling out salivary gland neoplasm. p63 and PSA were negative which ruled out prostatic carcinoma. Hence, a final diagnosis of primary cutaneous mucinous carcinoma was made.
It is still controversial whether this neoplasm has an eccrine or apocrine origin. In a histochemical study, it was suggested that primary cutaneous mucinous carcinoma undergoes eccrine differentiation based on findings such as abundant phosphorylase activity, limited nonspecific esterase, and acid phosphatase activities and strong positive reactions for a number of mitochondrial oxidative enzymes. In addition, by electron microscopy, the neoplastic cells of primary cutaneous mucinous carcinoma have shown to be similar to the dark cells of normal eccrine glands. There are few reports suggesting that mucinous carcinoma has apocrine differentiation, wherein tumor cells of mucinous carcinoma showed decapitation secretion in the neoplastic cells. Ultrastructural characteristics of neoplastic cells show apocrine differentiation such as electron dense bodies and vacuolization.
Primary cutaneous mucinous carcinoma has a relatively good prognosis with rare distant metastasis, but has risk of local recurrence (29.4%). Low metastatic potential has been attributed to the avascular characteristic of the tumor. Most metastases involve the regional lymph nodes and lungs, but rarely to bone marrow and parotid gland.
Current treatment remains excision with at least 1 cm margin since there is high local recurrence rate. The tumor is found to be resistant to chemotherapy and radiation. Patients with hormone receptor-positive tumourss may be treated using anti-estrogenic drugs like tamoxifen.
| Conclusion|| |
Detailed clinical history, examination, and histological findings aided by IHC can lead to proper diagnosis of primary cutaneous mucinous carcinoma. Differentiating it from metastatic mucinous carcinoma is important and helps in further management of the patient and prognosis.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Mnnaerki J, Ahmed S, Lee E. Primary mucinous carcinoma of the skin. Eplasty 2013;13:ic47.
Calonje E, Brenn T, Lazar A, Mckee PH. Tumors of the sweat glands. In: McKee PH, Editor. McKee's Pathology of the Skin with Clinical Correlations. 4th
ed. China: Saunders Elsevier; 2012. p. 1566-8.
Kim JB, Choi JH, Kim JH, Park HJ, Lee JS, Joh OJ, et al
. A case of primary cutaneous mucinous carcinoma with neuroendocrine differentiation. Ann Dermatol 2010;22:472-7.
Sistla R, Afroz T, Narayanswamy J. Primary mucinous adenocarcinoma of skin: myoepithelial cells are a clue to its diagnosis. Indian J Dermatol 2018; 63:165-7.
Krishnamurthy J, Saba F, Sunila. Primary mucinous cutaneous carcinoma of the skin: A rare tumor in the gluteal region. Indian J Pathol Microbiol 2009; 52:225-7.
Mardi K, Diwana VK. Primary cutaneous mucinous carcinoma: A rare entity. Indian Dermatol Online J 2011;2:82-4.
] [Full text]
Liu F, Zhang M, Sang H, Deng D. Primary mucinous carcinoma of the skin. Cutis 2014;94:E15-7.
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