Adnexal ยท EccrineICD-10 C44

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.

CurrentLast reviewed 26 April 2026

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 60โ€“70; 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+, CK20โˆ’, 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+, CK7โˆ’.
    • 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

  1. Snow SN et al. Mucinous eccrine carcinoma of the eyelid. Cancer; 1992.
  2. Kazakov DV et al. Mucinous carcinoma of the skin: clinicopathologic and immunohistochemical study. Am J Dermatopathol; 2007.

Spot a correction?

If any clinical statement, citation or link on this page needs updating, please email admin@skinoncology.net with the page name, the proposed correction and the supporting source.