8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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Primary Cutaneous Mucinous Carcinoma

Lisa Van Gemert, M4

Creighton University School of Medicine22 September, 2010

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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Case History

HPI: M 65, right upper arm growth

increasing in size

h/o sun exposure otherwise asymptomatic

PMH/FH/SocHx/Meds/All/ROS: Noncontributory

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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Physical Exam

Subcutaneous cystic nodule, 2-cm, right upper 

inner arm

Remainder of PE unremarkable

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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Impression

Pilomatricoma

No evidence of cancer 

E. Copcu : Pilomatricoma On The Lateral Canthus . The Internet Journal of Surgery. 2004 Vol. 5 -2

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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Procedure/ Gross

Full thickness, 3 cm excision length

Report of a case: Primary mucinous carcinoma of the skin. Dermatology Online Journal 14 (6): 4

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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H/E

Mucinous dermal tumor 

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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H/E

Islands of eosinophilic cells and duct-like structures within lakes of mucin

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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H/E

Mucin separated by fibrous septae

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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H/E

Tumor cells with round to cuboidal nuclei, prominent nucleolus,

vacuolated eosinophilic cytoplasm

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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Colloidal Iron

Positively staining (blue) stromal mucin

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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Mucicarmine

Positively staining (purple) stromal and glandular mucin.

Note positive intraluminal mucin (arrow)

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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CK 7

Neoplastic glands are positive.

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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Differential diagnosis

Metastatic mucinous carcinoma

Breast (Mammary mucinous carcinoma)

Chest wall, breast, axilla

GI Mucinous carcinoma (colon, stomach) Ant. abdominal wall

Lung, ovary, pancreas, salivary/lacrimal glands, urinarytract, paranasal sinuses

Mucin-producing BCC

Sebaceous cyst, hemangioma, lipoma, melanoma,epidermal inclusion cyst, chalazion, neuroma, Kaposi·s,

pilomatricoma, SCC

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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Diagnosis

Tumor summary

CK 7 Pos

CK 20 Neg

Mucicarmine Pos Colloidal Iron Pos

No clinical suspicion of metastasis

D x: Primary Cutaneous Mucinous Carcinoma aka ́ mucinous sweat gland carcinoma,µ

´mucinous eccrine carcinomaµ

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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Primary Cutaneous Mucinous

Carcinoma (PCMC)

Rare (~150 cases ) subtype of sweat gland tumor 

Epidemiology

Male (59%) > Female (41%) Age 8-87 y.o. (Mean 63)

Location

Eyelid ² 41%

Scalp ² 17% Face ² 14%

Axilla ² 9%

Chest/Abd. Wall ² 7%

Neck, vulva, extremity, canthus, groin, ear ² <5%

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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PCMC

Clinical Presentation

Painless

Frequently single, isolated

Papular or nodular  5 mm ² 120 mm

Red, pink, gray, blue, purple

+/- telangiectasia

Smooth, ulcerated, or crusted surface

Typically indolent course

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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PCMC

Work-up

Clinical

H/P ² breast examination

CBC, BMP, LFTs

Colonoscopy

Radiologic

CT chest/abdomen/pelvis

mammography

Barium enema

Pathologic Copious mucin secretion

Typically avascular 

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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PCMC

Immunohistochemistry

Positive:

Mucin - PAS, colloidal iron, mucicarmine, alcian blue at pH 2.5

LMW cytokeratin, vimentin, EMA Sometimes positive

S100, succinyl dehydrogenase, estrogen/progesteronereceptors, CEA

Negative

Mucin ² hyaluronidase, diastase, alcian blue at pH 0.4

CK20, P53, c-erbB-2

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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PCMC

Treatment

Wide local excision

Prophylactic LN dissection (axillary primary)

Prognosis

Recurrence common ² 29.4%

Metastasis ² 9.6%

Regional LNs

Bony invasion (7%)

Invasive mucinous carcinoma ² slower growth/lower metastasis vs. classic eccrine sweat gland tumors

Mortality ² 2%

8/8/2019 Primary Cutaneous Mucinous Carcinoma, A presentation by Lisa Van Gemert M4, Creighton University School of Medicine, Omaha, NE

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References

Primary Mucinous Carcinoma of the Skin: A Review. The InternetJournal of Oncology. 2005. Volume 2 Number 2.

Mucinous carcinoma of the skin, primary, and secondary: a

clinicopathologic study of 63 cases with emphasis on themorphologic spectrum of primary cutaneous forms: homologies with

mucinous lesions in the breast. American Journal of SurgicalPathology. 2005; 29(6): 764-82.

Mucinous carcinoma of the eyelid. An immunohistochemical study.

American Journal ofD

ermatopathology. 1995; 17: 494-498.

Primary mucinous carcinoma of the skin with metastases to the lymph

nodes. American Journal of Dermatopathology. 1985 Oct; 7(5): 461-9.