An Iranian male with syringoid eccrine carcinoma misdiagnosed as basal cell carcinoma: a case report
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Chinese-German Journal of Clinical Oncology June 2012, Vol. 11, No. 6, P365–P366 DOI 10.1007/s10330-011-0954-9 Melanoma skin cancers account for 4%–7% of all skin cancers, then non melanoma tumors for 93%–96%. Both basal cell carcinoma and squamous cell carcinoma repre- sent > 80% of non melanoma skin cancers whereas benign and malignant adnexal tumors represent only 1%–2%, in- cluding mesenchymal, fatty and vascular tumors. Adnex- al carcinoma of the skin drive from structures that have a common origin such as the apocrine and eccrine sweet gland, sebaceous glands and hair follicles. Malignant ad- nexal tumors are frequently located in the head and neck region but may appear on the fingers, toes, the trunk as well as the extremities [1–4] . Syringoid eccrine carcinoma is a very rare and uncommon diagnosed tumor thought to be derived from eccrine sweat apparatus. It locally inva- sive, destructive and often shows recurrence. Metastases in the regional lymph nodes are extremely rare as are dis- seminated metastasis [5] . Case presentation A 52 years old Iranian male was admitted to the De- partment of Dermatology, Shahid Sadoughi University in Yazd (Iran) in September 2009 for a 2 cm multinodular lesion on his scalp, frontoparietal area with an erythema- tous and partially eroded surfaces and covered by a sero- crust. The lesion was hard and attached to the hypoder- mis. The patient had history of irradiation to his scalp in adolescence. There was no evidence of lymph node en- largement. The lesion was small exophytic mass, yellow- ish in colour, and hard in consistency. Its size was about 2 × 1 cm in diameter. No enlargement of cervical lymph nodes was evident. The clinical impression was basal cell carcinoma or metastasis. Chest X-ray, abdominal sonog- raphy & CT-scan of head and neck revealed no remark- able findings suggesting visceral carcinoma. Laboratory data including tumor markers were within normal limits. The patients was in good condition and without subjec- tive difficulties (e.g. pain, fever and weight loss). The pa- tient underwent wide surgical excision and the raw area covered by split thickness skin graft. The specimen was fixed in 10% neutral formalin and embedded in paraf- fin. Five micron sections were stained with hematoxylin and eosin. Histologically, the tumor was located in the dermis and hypodermis and consisted of solid nests and small cords in a dense fibrocollagenous stroma. Solid tu- mor nests consisted of tumor cells which were basaloid and polymorphic, with hyperchromatic nuclei. A few mitotic figures were observed. A palisading arrangement of tumor cells, keratinous cysts, apocrine and follicular differentiation were not observed. There were no con- nection between the tumor nests and epidermis. The ex- An Iranian male with syringoid eccrine carcinoma misdiagnosed as basal cell carcinoma: a case report Binesh Fariba 1 , Akhavan Ali 2 , Kafaie Parichehr 3 , Navabii Hossein 4 1 Department of Pathology, Yazd Shahid Sadoughi University, Yazd, Iran 2 Department of Radiotherapy, Yazd Shahid Sadoughi University, Yazd, Iran 3 Department of Dermatology, Shahid Sadoughi University, Yazd, Iran 4 Medical Science, Yazd Shahid Sadoughi University, Yazd, Iran Received: 1 December 2011 / Revised: 20 January 2012 / Accepted: 25 February 2012 © Huazhong University of Science and Technology and Springer-Verlag Berlin Heidelberg 2012 Abstract Syringoid carcinoma (syringoid eccrine carcinoma, or eccrine epithelioma) is a rare cutaneous tumor with some controversy regarding its correct definition. This tumor shows a slow growth and has often been for many years, some decades before diagnosis. It may also be difficult to differentiate from its benign counterpart (syringoma) or other adnexal carcinoma and cutaneous metastasis. There have been limited case reports of syringoid carcinoma in foreign literatures but none from Iran. Here we report a case of syringoid carcinoma in a 52 year-old Iranian man. Syringoid eccrine carcinoma is a very rare and uncommon diagnosed tumor thought to be derived from eccrine sweat apparatus. It locally invasive, destructive and often shows recurrence. It may also be difficult to differentiate from metastatic adenocarcinoma. Key words adnexal carcinoma; syringoid; skin cancer Correspondence to: Binesh F. Email: [email protected]
Transcript of An Iranian male with syringoid eccrine carcinoma misdiagnosed as basal cell carcinoma: a case report
Chinese-German Journal of Clinical Oncology June 2012, Vol. 11, No. 6, P365–P366DOI 10.1007/s10330-011-0954-9
Melanoma skin cancers account for 4%–7% of all skin cancers, then non melanoma tumors for 93%–96%. Both basal cell carcinoma and squamous cell carcinoma repre-sent > 80% of non melanoma skin cancers whereas benign and malignant adnexal tumors represent only 1%–2%, in-cluding mesenchymal, fatty and vascular tumors. Adnex-al carcinoma of the skin drive from structures that have a common origin such as the apocrine and eccrine sweet gland, sebaceous glands and hair follicles. Malignant ad-nexal tumors are frequently located in the head and neck region but may appear on the fingers, toes, the trunk as well as the extremities [1–4]. Syringoid eccrine carcinoma is a very rare and uncommon diagnosed tumor thought to be derived from eccrine sweat apparatus. It locally inva-sive, destructive and often shows recurrence. Metastases in the regional lymph nodes are extremely rare as are dis-seminated metastasis [5].
Case presentation
A 52 years old Iranian male was admitted to the De-partment of Dermatology, Shahid Sadoughi University in Yazd (Iran) in September 2009 for a 2 cm multinodular lesion on his scalp, frontoparietal area with an erythema-
tous and partially eroded surfaces and covered by a sero-crust. The lesion was hard and attached to the hypoder-mis. The patient had history of irradiation to his scalp in adolescence. There was no evidence of lymph node en-largement. The lesion was small exophytic mass, yellow-ish in colour, and hard in consistency. Its size was about 2 × 1 cm in diameter. No enlargement of cervical lymph nodes was evident. The clinical impression was basal cell carcinoma or metastasis. Chest X-ray, abdominal sonog-raphy & CT-scan of head and neck revealed no remark-able findings suggesting visceral carcinoma. Laboratory data including tumor markers were within normal limits. The patients was in good condition and without subjec-tive difficulties (e.g. pain, fever and weight loss). The pa-tient underwent wide surgical excision and the raw area covered by split thickness skin graft. The specimen was fixed in 10% neutral formalin and embedded in paraf-fin. Five micron sections were stained with hematoxylin and eosin. Histologically, the tumor was located in the dermis and hypodermis and consisted of solid nests and small cords in a dense fibrocollagenous stroma. Solid tu-mor nests consisted of tumor cells which were basaloid and polymorphic, with hyperchromatic nuclei. A few mitotic figures were observed. A palisading arrangement of tumor cells, keratinous cysts, apocrine and follicular differentiation were not observed. There were no con-nection between the tumor nests and epidermis. The ex-
An Iranian male with syringoid eccrine carcinoma misdiagnosed as basal cell carcinoma: a case reportBinesh Fariba1, Akhavan Ali2, Kafaie Parichehr3, Navabii Hossein4
1 Department of Pathology, Yazd Shahid Sadoughi University, Yazd, Iran2 Department of Radiotherapy, Yazd Shahid Sadoughi University, Yazd, Iran3 Department of Dermatology, Shahid Sadoughi University, Yazd, Iran4 Medical Science, Yazd Shahid Sadoughi University, Yazd, Iran
Received: 1 December 2011 / Revised: 20 January 2012 / Accepted: 25 February 2012© Huazhong University of Science and Technology and Springer-Verlag Berlin Heidelberg 2012
Abstract Syringoid carcinoma (syringoid eccrine carcinoma, or eccrine epithelioma) is a rare cutaneous tumor with some controversy regarding its correct definition. This tumor shows a slow growth and has often been for many years, some decades before diagnosis. It may also be difficult to differentiate from its benign counterpart (syringoma) or other adnexal carcinoma and cutaneous metastasis. There have been limited case reports of syringoid carcinoma in foreign literatures but none from Iran. Here we report a case of syringoid carcinoma in a 52 year-old Iranian man. Syringoid eccrine carcinoma is a very rare and uncommon diagnosed tumor thought to be derived from eccrine sweat apparatus. It locally invasive, destructive and often shows recurrence. It may also be difficult to differentiate from metastatic adenocarcinoma.
Key words adnexal carcinoma; syringoid; skin cancer
Correspondence to: Binesh F. Email: [email protected]
366 www.springerlink.com/content/1613-9089
tent of tumor invasion could not be determined from the biopsy specimen, because the tumor had spread beyond the edge of excision (Fig. 1). The diagnosis was syringoid eccrine carcinoma.
Discussion
Syringoid eccrine carcinoma (SEC) was first described as eccrine epithelioma (Basal cell carcinoma with eccrine differentiation) by Freedman and Winkelman in 1969, and it is an extremely rare skin appendage tumor [6]. It usually affects subjects in the fourth to seventh decades of life and manifests as a solitary, firm nodule or plaque positioned on the scalp, face, or, more rarely, other sites [7]. Clinically our case mimics the most frequent appear-ance of SEC: a large sclerotic plaque on the scalp. He is the first case of SEC from Iran. The presented case had history of irradiation to his scalp in adolescence. We did not find any relationship between SEC and previous ra-diotherapy, although previous irradiation to the head and neck is a predisposing factor to some of head and neck malignancies, such as thyroid carcinoma [8]. According to this point our case is unique. Histologically SEC re-sembles syringoma by presenting with ductal, cystic and tad poled-shaped structure. SEC differs from syringoma by its cellularity and deep invasiveness. SEC needs to be differentiated from basal cell carcinoma (BCC) microcys-tic adnexal carcinoma (MAC) primary cutaneous adenoid cystic carcinoma (PCACC) and visceral carcinoma with skin metastasis. Distinction from BCC Should be straight forward in view of the lack of a palisading arrangement of tumor cells InSEC. MAC should be differentiated from SEC as MAC contains foci of eccrine and follicular dif-ferentiation and is composed of nests and strands of ba-saloid cells that form keratin filled cysts whereas SEC
does not usually form keratin filled cysts. PCACC usually shows a predominant cribriform pattern of tumor growth and histologic evidence of mucin production, which are lacking in SEC [9]. It may also be difficult to differentiate SEC from metastatic adenocarcinoma. The patient should be checked for visceral carcinoma carefully. The tumor grows slowly but is locally invasive and can metastasize to regional lymph nodes and subsequently to the bones and this has been reported in only one case [10]. Moy et al suggested that therapy of SEC is mostly surgical and nowadays Mohs micrographic surgery is the method of choice [11]. Subsequently our patient underwent wide lo-cal surgical excision followed by split thickness skin graft. Excellent aesthetic and functional result were obtained and recurrence was not detected after 3 months.
ConclusionIn conclusion, we emphasize that SEC is a rare primary
disease. Its clinical appearance is not well characterized. A complete local excision is effective in making both de-finitive diagnosis and treatment. It is rarely metastasizing to lymph nodes or distant organs and has a good progno-sis.
References
McKee Ph, Mariden R, Santacruz D. Tumors of the epithelial ap-pendages. In: McKee Ph, Mariden R, Santacruz D, editors. London: Mosby Wolfe, 1996. 151–157.Weedon D. Tumors of cutaneous appendage. In: Weedon D, editor. Skin pathology. London: Churchill Livingston, 1997. 713–758.Moulin G, Balme B, Thomas L. Tumeurs bénignes de l´épiderme. En-cycl Méd Chir. París: Elsevier, 1996. 17.Santacruz D. Farmer E, Hood A. Pathology of the skin. London: Appleton Lange; 1990. Tumors of sweat gland differentiation, 1996. 624–66.Elder D, Elenitsas R, Ragsdale BD. Syrigoid eccrine carcinoma. In: Lever F, Schaumburg-Lever G, editors. Histopathology of the skin. 8th eds. Philadelphia: Lippincott-Raven, 1997. 792–793.Freeman RG, Winkelmann RK. Basal cell tumor with eccrine differen-tiation (eccrine epithelioma). Arch Dermatol, 1969, 100: 234–242.Nishizawa A, Nakanishi Y, Sasajima Y, et al. Syringoid eccrine car-cinoma with apparently aggressive transformation: case report and review of the literature. Int J Dermatol, 2006, 45: 1218–1221.Fletcher CDM. Diagnostic histopathology of tumors. Philadelphia: Elsevier Health Sciences, 2007. 997.Nishizawa A, Nakanishi Y, Sasajima Y, et al. Syringoid eccrine car-cinoma with apparently aggressive transformation: case report and review of the literature. Int J Dermatol, 2006, 45: 1218–1221.Evans AT, Parham DM, Van Niekerk LJA. Metastasising eccrine syrin-gomatous carcinoma. Histopathology, 1995, 26: 185–187.Moy RL, Rivkin JE, Lee H, et al. Syringoid eccrine carcinoma. J Am Acad Dermatol, 1991, 24: 857–860.
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Fig. 1 Section shows skin tissue with ductal structures and small cords of cells in fibrous stroma (HE staining × 10)
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