Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obervation Letter
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Case Report
2019:85:2;171-174
doi: 10.4103/ijdvl.IJDVL_217_17
PMID: 29620041

Primary mucinous carcinoma of skin with a trichoadenomatous component: A rare case report

Athota Kavitha1 , Chennamsetty Kavya2 , Kovi Sneha1 , Chennamsetty Teja2
1 Dr. Paruchuri Rajaram Memorial Skin, Hair and Laser Centre, Guntur, Andhra Pradesh, India
2 Leelavathi Advanced Skin and Laser Centre, Guntur, Andhra Pradesh, India

Correspondence Address:
Chennamsetty Kavya
Leelavathi Advanced Skin and Laser Centre, Anna Srinivasarao Kalyanamandapam Road, Kothapeta, Guntur, Andhra Pradesh
India
Published: 04-Apr-2018
How to cite this article:
Kavitha A, Kavya C, Sneha K, Teja C. Primary mucinous carcinoma of skin with a trichoadenomatous component: A rare case report. Indian J Dermatol Venereol Leprol 2019;85:171-174
Copyright: (C)2019 Indian Journal of Dermatology, Venereology, and Leprology

Abstract


Primary mucinous carcinoma of the skin is a rare subtype of eccrine sweat gland tumors. Differentiating it from metastatic adenocarcinomas is important in the management of this condition. We report the case of a 55-year-old female presenting with a painless nodule, which was subsequently diagnosed as primary mucinous carcinoma of skin with a trichoadenomatous component. The possibility of a metastatic adenocarcinoma was ruled out by performing ultrasound abdomen, total body computed tomography, mammogram and colonoscopy.
Keywords: Cytokeratin-7, eccrine glands, mucinous carcinoma

Introduction

Primary mucinous carcinoma of the skin, a rare subtype of eccrine sweat gland tumor based on immunohistochemical and electron microscopic evidence, was first described by Lennox et al. in 1952[1] and was formally reviewed by Mendoza and Helwig in 1971.[2] Differentiating this condition from metastatic adenocarcinomas is crucial in the management of this condition.

Case Report

A 55-year-old female presented with a 5-year history of a painless nodule on the right zygomatic area. On examination, a skin colored nodule with a smooth surface without telangiectasia or ulceration, measuring about 5 × 5 mm, was noted [Figure - 1]. Regional lymph nodes were not palpable.

Figure 1: Skin-colored, smooth-surfaced nodule of 5 × 5 mm size on the right zygomatic area

Histopathological evaluation of the excised lesion revealed numerous keratinous cysts containing vellus hair in mucinous stroma within the upper dermis, separated from the mid and lower dermis [Figure - 2]a and [Figure - 2]b. Large pools of basophilic mucin separated by thin fibrovascular septa with floating islands of cohesive epithelial cells in solid and cribriform pattern were present in the mid dermis and subcutis. No significant mitoses were seen [Figure - 3]. Immunohistochemistry revealed cytokeratin (CK)-7, estrogen receptor and progesterone receptor positivity, smooth muscle actin, S-100P focal positivity and weak calponin positivity. CK-20, CDX2, anti-P63 were negative, favoring the diagnosis of primary mucinous carcinoma of skin [Figure - 4]. Investigations were done to rule out primary visceral malignancies; including ultrasound abdomen, total body computed tomography (CT), mammogram and colonoscopy, which were negative. The patient was treated surgically with excision of the tumor with a 1 cm margin and is on follow-up for the past 6 months, with no recurrence.

Figure 2:
Figure 3: Cords of epithelial cells in solid and cribriform pattern floating in basophilic mucin and seperated by fibrovascular septa (H&E, ×100)
Figure 4:

Discussion

Primary mucinous carcinoma of the skin is a very low-grade malignancy of the eccrine sweat glands.[3] Men are two times more commonly affected than women. The condition favors the head and neck region; eyelids being the most common site, followed by the scalp, face, axilla, vulva, chest, abdominal wall, neck, extremities, canthus, groin and ear.[3]

The tumor typically presents as an isolated, slow-growing, asymptomatic, painless, papular, nodular or subcutaneous growth ranging in size from 5 to 120 mm. The surface may be smooth, ulcerated or crusted and may or may not show telangiectasia.[4]

The histopathological features of primary mucinous carcinoma of skin include cords and nests of basaloid cells embedded in a pool of periodic-acid Schiff and colloid iron positive mucin, separated by thin fibrous septa.[5] Rosai hypothesized that the island of tumor cells floating in mucinous pools is due to ductal hyper-proliferation, leading to overproduction of mucin.[6] Mucin in primary mucinous carcinoma of skin is a non-sulfated mucoprotein, most likely sialomucin, and hence is hyalorunidase-resistant, alcian blue positive at pH 2.5 and negative at pH 0.4, in contrast to the gastrointestinal tumors which produce sulfamucins (alcian blue positive at pH 1.0 and 0.4). Eccrine origin of the tumor is supported by enzyme histochemistry with succinic dehydrogenase, lactic dehydrogenase and isocitric dehydrogenase positivity.[7]

Immunohistochemistry helps in differentiating primary mucinous carcinoma of the skin from metastatic adenocarcinomas from the breast, gastrointestinal tract, ovary, prostate, lung etc., This condition demonstrates consistently positive staining with low molecular weight cytokeratins and epithelial membrane antigens. P63/SMA staining helps in identifying the myoepithelial component that is present only in cutaneous tumors. Carcinoembryonic antigen (CEA) and S-100 protein expression is variable.[3],[8] Estrogen and progesterone receptor expression in primary mucinous carcinoma of skin is indistinguishable from mucinous adenocarcinoma arising in the breast with p-53 and c-erb-2 negativity.[3] Breast carcinomas are typically positive for GCDFP-15 and often for estrogen receptors, while negative for vimentin. Colonic carcinomas show prominent positivity for CEA and CK-20, while no staining is seen for estrogen receptor and vimentin.[9]

The clinical mimickers include sebaceous cyst, amelanotic melanoma, lymphoma and Merkel's tumors. Adenocystic basal cell carcinoma, poorly differentiated squamous cell carcinoma and metastatic adenocarcinomas are histopathological mimickers. Mucinous metastasis from colon and breast carcinoma to the skin are most commonly found on the anterior chest wall rather than the head and neck, where most primary skin tumors occur. Thorough evaluation of patients with breast examination, mammogram, total body CT scan and colonoscopy is mandatory to rule out visceral carcinomas and to diagnose primary carcinoma of skin.

Treatment of primary mucinous carcinoma of the skin is wide local excision with a 1 cm margin. Tumor is unresponsive to radiation or chemotherapeutic agents.[10] Regional lymph node resection is indicated only if nodes are clinically involved. Distant metastasis is rare because of the avascular nature.[11] However, local recurrences are common (29.4%) and hence close follow-up is warranted.

The presence of a follicular (trichoadenomatous) component in our case is intriguing. The presence of follicular/epithelial component within sweat gland tumors is usually suggestive of a mixed tumor (chondroid syringoma).[12] However, the histological features in our case do not favor a mixed tumor as the stromal component is most often chondromyxoid, hyalinized/fibrous, fatty, osteoid or may even be minimal or absent in a classical chondroid syringoma.[13] Perhaps follicular/epithelial induction differentiation within the primary mucinous carcinoma may explain the presence of a trichoadenomatous component within the tumor. We have not found such an occurrence previously described in the literature.

Declaration of patient consent

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

Acknowledgement

  1. Dr. P. Ramana Kumari, Consultant pathologist, RHP Lab, Guntur, Andhra Pradesh, India
  2. Dr. Sasi Kiran Athili, Dermatologist, Cosmetic surgeon and Dermatopathologist, Visakha Institute of Skin and Allergy, Visakhapatnam, Andhra Pradesh, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References
1.
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.
[Google Scholar]
2.
Mendoza S, Helwig EB. Mucinous (adenocystic) carcinoma of the skin. Arch Dermatol 1971;103:68-78.
[Google Scholar]
3.
Martinez S, Young S. Primary Mucinous Carcinoma of the Skin: A Review. Internet J Oncol 2004;2:2.
[Google Scholar]
4.
Scilletta A, Soma PF, Grasso G, Scilletta R, Pompili G, Tarico MS, et al. Primary cutaneous mucinous carcinoma of the cheek. Case report. G Chir 2011;32:323-5.
[Google Scholar]
5.
Bellezza G, Sidoni A, Bucciarelli E. Primary mucinous carcinoma of the skin. Am J Dermatopathol 2000;22:166-70.
[Google Scholar]
6.
Rosai J. Ackerman's Surgical Pathology. 7th ed. St. Louis, MO: Mosby; 1989. p. 1232-3.
[Google Scholar]
7.
Headington JT. Primary mucinous carcinoma of skin: Histochemistry and electron microscopy. Cancer 1977;39:1055-63.
[Google Scholar]
8.
Carson HJ, Gattuso P, Raslan WF, Reddy V. Mucinous carcinoma of the eyelid. An immunohistochemical study. Am J Dermatopathol 1995;17:494-8.
[Google Scholar]
9.
Lagendijk JH, Mullink H, van Diest PJ, Meijer GA, Meijer CJ. Immunohistochemical differentiation between primary adenocarcinomas of the ovary and ovarian metastases of colonic and breast origin. Comparison between a statistical and an intuitive approach. J Clin Pathol 1999;52:283-90.
[Google Scholar]
10.
Yeung KY, Stinson JC. Mucinous (adenocystic) carcinoma of sweat glands with widespread metastasis. Case report with ultrastructural study. Cancer 1977;39:2556-62.
[Google Scholar]
11.
Martinez S, Young S. Primary mucinous carcinoma of the skin: A review. Internet J Oncol 2004;2:2.
[Google Scholar]
12.
Obaidat NA, Alsaad KO, Ghazarian D. Skin adnexal neoplasms – Part 2: An approach to tumours of cutaneous sweat glands. J Clin Pathol 2007;60:145-59.
[Google Scholar]
13.
Kakinuma H, Miyamoto R, Iwasawa U, Baba S, Suzuki H. Three subtypes of poroid neoplasia in a single lesion: Eccrine poroma, hidroacanthoma simplex, and dermal duct tumor. Histologic, histochemical, and ultrastructural findings. Am J Dermatopathol 1994;16:66-72.
[Google Scholar]

Fulltext Views
326

PDF downloads
92
Show Sections