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| | | Year : 2009 | Volume : 52 | Issue : 2 | Page : 225-227 | | | Primary mucinous carcinoma of the skin: A rare tumor in the gluteal region | | | Jayashree Krishnamurthy, Firdos Saba, Sunila
Department of Pathology, JSS Medical College, Mysore, India
Click here for correspondence address and email | |      | |  Abstract | | | Primary mucinous carcinoma (PMC) of the skin is a rare adnexal tumor of sweat gland origin. A case report is presented of a 50-year-old female who presented with a gluteal mass, which was diagnosed as an injection abscess. Following incision and drainage, the incision site persisted as a non-healing ulcer. An edge biopsy of the lesion revealed mucinous carcinoma of the skin. Investigations excluded the possibility of a metastatic mucinous carcinoma. Thus, the lesion in the gluteal region was diagnosed as PMC of the skin, a rare site of occurrence. Keywords: Adnexal neoplasm, colloidal iron, mucinous carcinoma, myoepithelial component, periodic acid schiff How to cite this article:
Krishnamurthy J, Saba F, Sunila. Primary mucinous carcinoma of the skin: A rare tumor in the gluteal region. Indian J Pathol Microbiol 2009;52:225-7 |
How to cite this URL:
Krishnamurthy J, Saba F, Sunila. Primary mucinous carcinoma of the skin: A rare tumor in the gluteal region. Indian J Pathol Microbiol [serial online] 2009 [cited 2014 Mar 5];52:225-7. Available from: http://www.ijpmonline.org/text.asp?2009/52/2/225/48926 |
| Introduction | |  |
Primary mucinous carcinoma (PMC) of the skin is a rare adnexal neoplasm with sweat gland differentiation. [1] It has a relatively good prognosis, with rare distant metastases but a high recurrence rate and occasional regional lymph node metastasis. [2] Mucinous carcinoma can occur in non-cutaneous visceral sites and may metastasize to the skin. Thus, it is important to exclude the possibility of a cutaneous metastasis of mucinous carcinoma before diagnosing a PMC. [1]
| Case Report | | |
A 50-year-old female presented with a gluteal mass, which was diagnosed as an injection abscess. Following incision and drainage, she presented with a non-healing ulcer, which persisted for a 4-month duration. On examination, the ulcer measured 5 × 6cm. It had raised edges, indurated margins and had serous discharge. An edge biopsy was taken for histopathological examination.
Pathological findings
Macroscopically, the specimen consisted of skin with subcutaneous tissue measuring 4 × 3 × 3cm.
Microscopically, the sections revealed a tumor in the dermis composed of nests of cells arranged in irregular tubular and glandular patterns, with few showing cystic dilatation. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa [Figure 1].
The mucin was periodic acid schiff (PAS) positive and diastase resistant [Figure 2]. With alcian blue, the mucin was positive at pH 2.5 and negative at pH 1.0 and pH 4.0, indicating that it was a non-sulfated sialomucin, an epithelial mucin. It was also positive for colloidal iron.
Further investigations, including colonoscopy, ultrasonographic examination of the abdomen and computerized tomography (CT) scan of the chest, abdomen and pelvis were found to be normal. Immunostains CK7 and CK20 to rule out metastatic colon cancer, P63 and CK5/6, the myoepithelial markers that favor a diagnosis of PMC, were not performed due to the non-availability of the facility. Thus, the lesion in the skin was reported as PMC of the skin.
| Discussion | | |
PMC of skin is an uncommon slow growing tumor [3] with frequent local recurrence (29.4%) [4,5] but has a low rate of metastasis (9.6%). [4] It is slightly more common in men (58.8%) than in women (41.2%) and typically affects patients in the fifth to seventh decades of life. [5]
Mucinous carcinoma most commonly arises in the head and neck region, with the eyelid (41%) and the scalp (17%) being the most common sites. [4] It also affects the face (14%), axilla (9%), vulva (4%), chest/abdominal wall (7%), neck (2%), extremity (2%), canthus (2%), groin (1%) and ear (1%). [4] It usually presents as a painless nodule and the surface may be smooth, ulcerated or crusted. [4],[5]
The present case was a 50-year-old female who presented with a non-healing ulcerated lesion in the gluteal region, a rare site of occurrence that has not been reported to date.
Clinically, the differential diagnoses include epidermal inclusion cyst, lipoma, hemangioma, cystic basal cell carcinoma, melanoma, squamous cell carcinoma, sebaceous cyst and metastatic adenocarcinoma. [4],[5] Of these, the most important to consider is the metastasis of mucinous carcinoma to the skin from another site. [4]
The mucinous carcinomas that are known to metastasize to the skin are from the gastrointestinal tract, ovary, breast, respiratory tract, salivary glands, lacrimal glands, urinary tract, prostate or from the paranasal sinuses. [4]
Mucinous carcinomas are dermal tumors that sometimes extend into the subcutis and deeper tissue. [3] Histologically, multiple small monomorphic epithelial cells arranged in cords, lobules, cribriform or solid patterns are seen floating in pools of mucin. [5],[6] The epithelial cells are polygonal with moderate to abundant eosinophilic cytoplasm. The nuclei are usually vesicular with inconspicuous nucleoli and few, if any, show mitoses. [5] Microscopic examination of the present case showed columnar cells that had hyperchromatic nuclei. The tumor cells were arranged in irregular tubular and glandular patterns, with few showing cystic dilatation.
Kazakov et al . [7] have opined that most lesions originated from in situ lesions, which defines the neoplasm as primary cutaneous, but its absence does not exclude the diagnosis. They have also suggested that a full clinical assessment is essential for such neoplasms. [5]
Qureshi et al. [6] have indicated the presence of the myoepithelial component in PMC to distinguish it from metastatic mucinous carcinoma from either breast or sites elsewhere.
The mucin shows PAS positivity and stains with mucicarmine and colloidal iron. It is hyaluronidase resistant and sialidase labile, indicating that it is a sialomucin. This feature assists in differentiating this tumor from a metastatic mucinous carcinoma, which it may closely mimic. [3] In the present case, the mucin showed positivity for PAS and colloidal iron and it was diastase resistant.
Electron microscopically, the tumor is composed of peripheral dark cells and inner pale cells. [3],[4] These dark cells resemble the cells of the eccrine secretory coil, lending support to the tumor's eccrine origin. [4]
PMCs rarely metastasize. Most metastases are seen to involve the regional lymph nodes, lung and, rarely, parotid gland. [4],[8] Aggressive bony invasion has also been reported (7%) but is uncommon. [4]
| Conclusion | | |
PMC of the skin is a rare tumor of sweat gland origin. It has a relatively good prognosis, with rare distant metastasis. Extensive investigations are required to exclude the possibility of a non-cutaneous visceral primary tumor. The rarity and the infrequent location in the gluteal region necessitates a high index of suspicion to make an accurate diagnosis.
| References | | |
| 1. | 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.  | | 2. | Klein W, Chan E, Seykora JT. Tumors of the epidermal appendages. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, editors. Lever′s Histopathology of skin. 9 th ed. Lippincott Williams and Wilkins; 2005. p. 912. | | 3. | Weedon D, Strutton G. Tumors of cutaneous appendages. In: Skin pathology. 2 nd ed. St Louis: Elsevier Ltd; 2002. p. 885. | | 4. | Martinez SR, Young. Primary mucinous carcinoma of the skin: A review. Int J Oncol 2005;2:2. Available from: http://www.ispub.com/. | | 5. | Reid-Nicholson M, Iyengar P, Friedlander MA, Lin O. Fine needle aspiration biopsy of primary mucinous carcinoma of the skin. Acta Cytol 2006;50:317-22. [PUBMED] | | 6. | Qureshi HS, Salama ME, Chitale D, Bansal I, Ma CK, Raju U, et al . Primary cutaneous mucinous carcinoma: Presence of myoepithelial cells as a clue to the cutaneous origin. Am J Dermatopathol 2004;26:353-8. [PUBMED] [FULLTEXT] | | 7. | Kazakov DV, Suster S, Leboit PE, Calonje E, Bisceglia M, Kutzner H, et al . Mucinous carcinoma of the skin, primary, and secondary: A clinicopathologic study of 63 cases with emphasis on the morphologic spectrum of primary cutaneous forms: Homologies with mucinous lesions in the breast. Am J Surg Pathol 2005;29:764-82. [PUBMED] [FULLTEXT] | | 8. | Jih MH, Friedman PM, Kimyai-Asadi A, Goldberg LH. A rare case of fatal primary cutaneous mucinous carcinoma of the scalp with multiple in-transit and pulmonary metastases. J Am Acad Dermatol 2005;52 S76-80. |
Correspondence Address:
Jayashree Krishnamurthy
Door number 1670, 7th cross, Narayan Shasthri road, Mysore - 570 004
India
DOI: 10.4103/0377-4929.48926 PMID: 19332921
[Figure 1], [Figure 2] | |
| This article has been cited by | | 1 | Metastatic mucinous cutaneous carcinoma: Prolonged response to Systemic chemotherapy | | | Pachón, V., López, M., Vaz, M.A., Grande, E., Blanco, M.J. | | European journal of Clinical and Medical Oncology. 2011; 3(2): 82-84 | | [Pubmed] | | | 2 | Primary mucinous carcinoma of skin: Report of a case | | | Wu, S.-C., Lin, S.-P., Xie, X.-M. | | Chinese Journal of Pathology. 2011; 40(3): 196-197 | | [Pubmed] | |
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