skin adnexal neoplasm - suny downstate medical center neoplasm.pdf · skin adnexal neoplasms—part...

Skin adnexal neoplasm

Volodymyr Labinskyy, MD SUNY Downstate

12/05/2013

www.downstatesurgery.org

• 47 y.o. M c/o growing mass on the left cheek for the past

8 years

• PMH: none

• PSH: none

• Allergies: NKDA

• Home meds: denies

• Family Hx: non significant

• Biopsy was performed 2 weeks prior to hospitalization

and revealed sebaceous carcinoma with squamous cell

carcinoma features.


10/22/13 www.downstatesurgery.org

Speciment: A tan-yellow, lobulated, soft tumor measuring 3 x 3 x 3 cm. The deep aspect of the tumor is covered by a thin (less

than 0.1 cm) intact membrane-like tissue.


Left cheek mass, excision: Squamous cell carcinoma (3 cm) with focal sebaceous differentiation and extensive spindle cell sarcomatoid component. Closest margin is deep, less than 0.5 mm. Other margins = 3 (superior) - 8 mm.


Skin Adnexal Neoplasm


Adnexal tumors

• Hair follicles

• Sebaceous glands

• Sweat glands

– Eccrine

– Apocrine


WHO histological classification of appendageal

tumours


TNM classification of skin appendageal carcinomas


NEW AND UNDERRECOGNIZED ENTITIES

• Endocrine Mucin-producing Sweat Gland Carcinoma, described

by Flieder in 1997, is a rare low-grade sweat gland carcinoma, with

few reported cases in the literature.

• Congenital Panfollicular Nevus. In 2005, Fin and Argenyi reported

a case of an unusual congenital hamartoma.

• The Cribriform Pattern—Primary Cutaneous Cribriform

Apocrine Carcinoma. Described by Requena et al in 1998, this is a

rare nonencapsulated, primarily dermal tumor with prominent

cribriform pattern.

• Spindle Cell Predominant Trichodiscoma/Neurofollicular Hamartoma described Recently by Kutzner et al.


Differential diagnosis IMMUNOHISTOCHEMISTRY

FIGURE 1. Strong calretinin

immunostaining of tricholemmal

cyst.

FIGURE 2. Calretinin

immunostaining of inner layers of

outer root sheath of the hair follicle.

Staining of superficial basal cell

carcinoma and eccrine glands is

present.

Calretinin is calcium-binding

protein, within normal skin

structures and cutaneous adnexal

proliferations.


Basal Cell Carcinoma Versus Trichoepithelioma • Recently, D2-40 (podoplanin), a lymphatic marker, was proposed as a potential reliable

immunohistochemical marker in this scenario.

Microcystic Adnexal Carcinoma Versus Basal Cell

Carcinoma • BerEP4, an epithelial marker was recently described to reliably differentiate between

MAC from basal cell carcinoma

Morpheaform Basal Cell Carcinoma Versus Desmoplastic

Trichoepithelioma

• p75NTR (neurotrophin receptor) and PHLDA1 (pleckstrin homology-like domain, family

A, member 1) have the benefit of diffuse staining of DTE studied by Krahl and

Sellheyer


Abbreviations: CI, confidence interval; Freq, frequency; ICD-O-3, International Classification of Diseases for Oncology, Third Edition; IRR, incidence rate ratio; NOS, not otherwise specified; NR, not reported (statistic not presented owing to less than 10 cases); SEER, Surveillance, Epidemiology, and End Results. aExcludes Alaska registry and American Indian, Alaska Native, and unknown/other race. bRates are per 1 million person-years and age-adjusted to the 2000 US standard population (19 age groups20).


Age-specific cutaneous appendageal carcinoma incidence rates (IRs), in Surveillance, Epidemiology, and End Results 16 from 2001 through 2005.18 A, All appendageal carcinomas (total), apocrine-eccrine tumors, sebaceous carcinoma, and skin appendage carcinoma, not otherwise specified (NOS); B, apocrine-eccrine carcinoma subtypes including porocarcinoma, eccrine carcinoma, mucinous carcinoma, and adenoid-cystic carcinoma; C, apocrine-eccrine carcinoma subtypes including microcystic adnexal carcinoma, hidradenocarcinoma, and other apocrine-eccrine carcinoma (including apocrine carcinoma, spiradenocarcinoma, digital papillary carcinoma, and malignant mixed tumor).


Abbreviations: NOS, not otherwise specified; SEER, Surveillance, Epidemiology, and End Results. aExcludes Alaska registry and American Indian, Alaska Native, and unknown/other race. b Extremities include both upper limb and lower limb combined. c Includes tumor types below and subcategory “other apocrine-eccrine tumors.”


Sebaceous Gland Carcinoma

• Is a rare aggressive cutaneous malignancy originates from the

meibomian or Zeis glands

• The ocular region, particularly the upper eyelid, is the most common

location for sebaceous carcinomas to develop.

• Noncontiguous multicentric histologic patterns and pagetoid spread

of tumor cells are common and may contribute to the high

recurrence rate of sebaceous carcinoma.


Sebaceous carcinoma is the

fourth most common eyelid

malignancy, representing 0.2 to

5.5% of all eyelid cancers.

Ocular sebaceous carcinoma

typically affects adults in their

sixth to eighth decades of life,

and is more common in

women.


Extraocular sebaceous

carcinoma most commonly

arises in the head and neck

region, occurs with greatest

frequency in older adults, and

does not exhibit a predilection

for men or women.

Regardless of location,

sebaceous carcinoma is an

aggressive malignancy, with

local recurrence rates after

Mohs surgery reported to be 9

to 36%.


Muir-Torre syndrome

• Autosomal dominant condition characterized by sebaceous tumors, multiple keratoacanthomas especially in younger patients in sunprotected areas, and visceral malignancy, particularly colorectal, endometrial, urological, and upper GI tract.

• Defective DNA mismatch repair genes, resulting in microsatellite instability, are implicated in Muir-Torre syndrome.

Sebaceous adenoma in a patient with Muir-Torre

syndrome.


Brooke-Spiegler Syndrome

• Autosomal dominant disease

characterized by the presence of

spiradenomas, cylindromas,

spiradenocylindromas, and

trichoepitheliomas

• Patients present with multiple papules

and nodules favoring the scalp, face,

and preauricular areas

• Salivary gland tumors affecting the

parotid and submandibular glands can

occur

A, Cylindroma—firm nodule on scalp. B, Histology of cylindroma—mosaic or “jigsaw puzzle pattern” of basaloid tumor islands.


Cowden Syndrome

• Multiple hamartoma syndrome, is

an autosomal dominant

genodermatosis.

• Trichilemmomas are a hallmark of

this disease

• Other cutaneous lesions are

multiple sclerotic fibromas,

palmoplantar keratoses, oral

papillomatosis, and “scrotal”

tongue.

• Cowden syndrome is linked with

mutations involving the tumor

suppressor gene PTEN.

• Extracutaneous lesions include

neoplasms of the breast, thyroid

gland, uterus, CNS, and GI tract


Treatment

• The mainstay of treatment of skin adnexal tumors is adequate excision, with monitoring of margins with both permanent and frozen sections.

• Some patients with perineural invasion could be treated with postoperative adjuvant radiotherapy after excision of the primary tumor.

• Topical antimetabolites are used in some cases.


Abbreviations: NOS, not otherwise specified; NR, not reported (10 cases); SEER, Surveillance, Epidemiology, and End Results; SR, survival rate. a The period of survival was from the date of diagnosis to the date of last contact, death, or December 31, 2005. b Excludes Alaska registry and American Indian, Alaska Native, and unknown/other race. The rest of California (excluding San Francisco/San Jose-Monterey/Los Angeles), Kentucky, Louisiana, New Jersey contribute cases for diagnosis years 2000 through 2004. The remaining 12 SEER areas contribute cases for the entire period of 1992 through 2004. c Includes tumor types below and subcategory “other apocrine-eccrine tumors.” Porocarcinoma and eccrine carcinoma are not included in survival analyses because of their recent International Classification of Diseases coding. d A total of 219 tumors were unstaged.


References

1. Aris Ntomouchtsis, et al. Adnexal Skin Carcinomas of the Face. J Craniofac Surg 2009;20: 134Y137

2. S. M. Ali, O. P. Sangueza. What is New in Adnexal Tumors of the Skin? Adv Anat Pathol

2013;20:334–346

3. P. W. Blake et al. Cutaneous Appendageal Carcinoma Incidence and Survival Patterns in the United

States. Arch Dermatol. 2010;146(6):625-632

4. S. R. Martinez et al. Rare Tumors Through the Looking Glass. Arch Dermatol. 2011;147(9):1058-

1062

5. K O Alsaad, N A Obaidat, D Ghazarian. Skin adnexal neoplasms—part 1: An approach to tumours

of the pilosebaceous unit. J Clin Pathol 2007;60:129–144.

6. A Neil Crowson et al. Malignant adnexal neoplasms. Modern Pathology (2006) 19, S93–S126


A 75-year-od man has a newly noted, raised 1.5 cm pearly

nodule with surface teleangiectasia on the cheek. What is

the next most appropriate step in his care?

A. Punch biopsy

B. Topical imiquimod

C. Curettage

D. Surgical excision

E. Radiation therapy


A fair-skinned 68 year old woman has a sharply

demarcated 2 cm ulcerated skin lesion in an old burn scar

on her forearm. What is the most appropriate treatment for

this patient?

A Topical chemotherapy

B Topical biologic therapy

C Surgical excision with frozen section

D Mohs micrographic surgery

E Radiotherapy


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