Case 12 Female 71. Longstanding nodule from shoulder recently enlarging. Clinically sebaceous cyst
Case 12 Female 71. Longstanding nodule from shoulder recently enlarging. Clinically sebaceous cyst
Case 12 Malignant areas with marked atypia
Case 12: Malignant spiradenoma (spiradenocarcinoma), poorly differen ated, arising in a benign spiradenoma – a rela vely rare occurence
Spiradenocarcinoma:
spiradenocarcinoma either 1) well differen ated and low grade resembling spiradenoma and retain lobularity, but no lymphocytes or two cell pa ern, or 2) Poorly differen ated with no obvious spiradenomatous differen a on & rely on benign counterpart for diagnosis (as in this case) & Shows typical features of a malignant neoplasm:
spiradenoma • infiltra ve border • lacks organised structure of spiradenoma • Lacks clearly demarcated two cell popula on of spiradnoma
Case 12: Malignant spiradenoma (spiradenocarcinoma), poorly differen ated, arising in a benign spiradenoma – a rela vely rare occurence
spiradenocarcinoma • typical features of a malignant neoplasm:
• Larger cells , overlapping nuclei.
• Increased mito c ac vity and cellular pleomorphism.
spiradenoma • Necrosis may occur (but can get degnera ve change in spiradenoma mimicking necrosis)
• O en long history, elderly, at any site
Spiradenoma Spiradenocarcinoma
Case 12: Malignant spiradenoma (spiradenocarcinoma), poorly differen ated, arising in a benign spiradenoma – a rela vely rare occurence
•72 pa ents in metaanlysis •35 pa ents with no distant metastasis, local resec on resulted in100% disease-free survival. •12 had lymph node metastases but no distant metastases • Of 7 pa ents with lymph node but no distant metastasis treated with surgical resec on and lymph node dissec on, 6 pa ents remained disease-free at final follow-up evalua on
•24 cases with confirmed distant metasta c disease •median survival 16 mpnths • pa ent survival did not significantly differ between local resec on and surgery with adjuvant chemoradiotherapy
CONCLUSIONS: An aggressive surgical approach is supported in the absence of metastasis. When lymph nodes are not clinically involved, sen nel node may have a role followed by lymph node dissec on in pa ents with a posi ve node.
Andreoli MT, Itani KM. Malignant eccrine spiradenoma: a meta-analysis of reported cases. Am J Surg. 2011 May;201(5):695-9
Spiradenoma (eccrine spiradenoma)
• Well circumscribed dermal or some mes dermal and subcutaneous nodule
• may have mul ple lobules and therefore may be incompletely excised. • If excised completely do not recur
Spiradenoma (eccrine spiradenoma)
Intermingled dual cell popula on of epithelial cells (corresdpond to ductal and myoepithelial cells)
• Striking “peppering” with lymphocytes.
• Rounded larger lobules lack “jigsaw puzzle” pa ern of cylindroma but have eosinophilic PAS posi ve basement membrane material around lobules
• Ducts seen in most cases
• Generally lack mito c ac vity or cellular pleomorphism.
Spiradenoma (eccrine spiradenoma)
• O en very vascular with haemorrhage
• Oedema in “lymphnagiomatous” variant (as in areas of this case)
• Tumours showing a combina on of spiradenomatous and cylindromatous features are common (spiradenocylindroma or cylindrospiradenoma).
• Usually solitary , o en blue, 3mm -50mm dermal nodule, especiially head and neck • Mul ple lesions on the face and scalp in the inherited autosomal dominant Brooke- Spielger syndrome (with cutaneous cylindromas, spiradenomas, spiradenocylindromas and trichoepitheliomas) and a germline muta on in the CLYD gene.
Spiradenoma (eccrine spiradenoma) differen al diagnosis
• Very characteris c morphology so few close mimics
• Cylindroma
• Other benign adnexal neoplasms
• Malignant spiradnoma: (NB can see pseudoinvasion inside a benign spiradenoma - pictured - but not beyond the lesion unless trauma sed)
Spiradenoma (eccrine spiradenoma) resemblance to eccrine sweat ducts
Case 12: Malignant spiradenoma (spiradenocarcinoma)
differen al diagnosis primary or secondary adenoid cys c carcinoma
Case 12: Malignant spiradenoma differen al diagnosis: porocarcinoma
Ulcerated porocarcinoma – strands of tumour “raining down” porocarcinoma – maligant duct lumen
porocarcinoma – normal pre-exis ng sweat duct porocarcinoma – DPAS highlights malignant duct cu cle surrounding central mucin globule
porocarcinoma – CEA highlights malignant duct Case 12: Malignant spiradenoma differen al diagnosis: Metastasis – o en central necrosis, immunohistochemical proflie
Cytokera n 15, podoplanin (D2-40) and p63 posi ve in most primary skin adnexal neoplasms and nega ve in metasta c adenocarcinoma
• Plaza JA, Ortega PF, Stockman DL, Suster S. Value of p63 and podoplanin (D2-40) immunoreac vity in the dis nc on between primary cutaneous tumors and adenocarcinomas metasta c to the skin: a clinicopathologic and immunohistochemical study of 79 cases. J Cutan Pathol. 2010 Apr;37(4): 403-10. • Liang H, Wu H, Giorgadze TA, Sariya D, Bellucci KS, Veerappan R, Liegl B, Acs G, Elenitsas R, Shukla S, Youngberg GA, Coogan PS, Pasha T, Zhang PJ, Xu X. Podoplanin is a highly sensi ve and specific marker to dis nguish primary skin adnexal carcinomas from adenocarcinomas metasta c to skin. Am J Surg Pathol. 2007 Feb;31(2):304-10. • Mahalingam M, Nguyen LP, Richards JE, Muzikansky A, Hoang MP. The diagnos c u lity of immunohistochemistry in dis nguishing primary skin adnexal carcinomas from metasta c adenocarcinoma to skin: an immunohistochemical reappraisal using cytokera n 15, nes n, p63, D2-40, and calre nin. Mod Pathol. 2010 May;23(5):713-9. Cytokera n 15, podoplanin and p63 posi ve in most primary skin adnexal neoplasms and nega ve in metasta c adenocarcinoma
Cytokera n 15 Expressed in sebaceous glands, outer root sheath of the hair follicle including bulge area, adult epidermis most specific for primary skin adnexal carcinomas (98%)
P63 member of p53 gene family transcrip on factor for maintaining epithelial stem cell prolifera on normal expression basal and lower part of epidermis, peripheral cells of eccrine ducts, apocrine glands, basal cells of sebaceous glands 91-100% of adnexal neoplasms, 14% of metasta c adenocarcinomas most sesn
Podoplanin : strongly posi ve in normal basal cells of sebaceous glands basal layer of outer root sheath of hair follicles including the bulge area basal layer of epidermis stains 0-4% of metasta c adenocarcinomas only 86% of primary skin neoplasms (but 65% only focally posi ve) Malignant stained more than benign 40% basal cell carcinomas posi ve for podoplanin
Posi vity for all 3 makers very good evidence for primary skin adnexal carcinoma P63 expression: D2-40 (podoplanin) normal skin normal skin may also show staining of base of epidermis (not seen here) Metasta c colorectal adenocarcinoma P63 nega ve
CK7 nega ve D2-40 (podoplanin) nega ve (stains adnexal structures) Mixed tumour (chondroid syringoma) Cytokera n 7 Cytokera n 20 P63 normal skin P63 mixed tumour D2-40 (podoplanin) D2-40 (podoplanin) mixed normal skin tumour
D2-40 (podoplanin) sebaceoma