Primary cutaneous mucinous carcinoma
Primary mucinous carcinoma of skin (PMCS); colloid carcinoma of skin
Primary cutaneous mucinous carcinoma is a rare low-grade adnexal carcinoma with eccrine differentiation, characterised histologically by clusters of tumour cells "floating" in pools of extracellular mucin. It typically presents on the face โ particularly the eyelid, scalp and cheek โ of older adults as a slowly enlarging, gelatinous, blue-grey or skin-coloured nodule. The single most important diagnostic step is to exclude metastatic mucinous adenocarcinoma from the breast, gastrointestinal tract or other primary, since the histology can be indistinguishable. Once a primary cutaneous origin is confirmed, the prognosis is excellent and Mohs micrographic surgery is the treatment of choice.
Clinical features
- Slow-growing, gelatinous-feeling, blue-grey, translucent or skin-coloured nodule.
- Most common on the face (eyelid in particular ~40%), scalp, neck and trunk.
- Median age 60โ70; M>F (slight).
- Often present for years; may be cystic on palpation.
- Differential: epidermal cyst, BCC, Merkel cell carcinoma, hidradenoma.
Histology & rule out metastasis
- Nests, cords or sheets of monomorphic epithelial cells suspended in pools of basophilic extracellular mucin, divided by delicate fibrous septae.
- Tubular and cribriform architecture often present.
- Low-grade nuclei; mitoses uncommon.
- Immunohistochemistry โ primary cutaneous: CK7+, CK20โ, ER+, PR+, GCDFP-15+, p63+ (myoepithelial component at periphery).
- Critical step: exclude metastatic mucinous carcinoma:
- Breast โ same IHC profile; clinical examination, mammography and breast ultrasound mandatory.
- Colorectal โ CK20+, CDX2+, CK7โ.
- Lung, pancreas, ovary, prostate.
- The presence of an in-situ component or focal apocrine differentiation favours primary cutaneous origin.
Staging workup
- Detailed history (any prior cancer, breast lump, change in bowel habit, weight loss, etc.).
- Full skin examination including breast and oral mucosa.
- Mammography (women) and breast ultrasound; consider in men with suspicious lesions.
- CT chest/abdomen/pelvis to exclude an internal primary, especially for tumours not on the face.
- Discuss at skin cancer MDT (and breast MDT if eyelid lesion in a woman).
Management
- Mohs micrographic surgery is the treatment of choice โ preserves cosmesis (frequently periorbital) and minimises recurrence.
- Wide local excision with 1 cm margins is acceptable where Mohs is unavailable.
- Sentinel lymph node biopsy not routinely indicated; nodal metastasis rare.
- Adjuvant radiotherapy reserved for incomplete margins or recurrent disease.
- Hormone-receptor-directed therapy may be considered for the rare metastatic case (off-licence).
Prognosis
Excellent โ local recurrence after Mohs <10%; metastasis <5%. The principal long-term concern is misdiagnosis as benign or as metastatic carcinoma at presentation. Standard skin-cancer follow-up (annual for 5 years) is appropriate after complete excision.
References
- Snow SN et al. Mucinous eccrine carcinoma of the eyelid. Cancer; 1992.
- Kazakov DV et al. Mucinous carcinoma of the skin: clinicopathologic and immunohistochemical study. Am J Dermatopathol; 2007.
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