Deba P Sarma and James Shehan publication: Concurrent Merkel cell carcinoma and Bowen's disease of the thigh

8/2/2019 Deba P Sarma and James Shehan publication: Concurrent Merkel cell carcinoma and Bowen's disease of the thigh.

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Publication, 2006-11

Sarma DP, Wang B, Shehan J, Linder-Stephenson L (2007). Concurrent Merkel cell

carcinoma and Bowen's disease of the thigh. TheInternet J Dermatol 5(2). Indexed by Google

Scholar.

Deba P Sarma, MD, Dermatopathologist, Lakeside Hospital James Shehan, MD, Dermatologist, Bergan Mercy Hospital

Omaha

http://www.ispub.com/ostia/index.php?xmlFilePath=journals/ijd/vol5n2/bowen.xml




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Pan Z, Albertson D, Bhuller A, Wang B, Shehan JM, Sarma DP. (2008) Angiosarcoma of thescalp mimicking a sebaceous cyst. Dermatolgy Online J 14 (6): 13. PMID: 18713594

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Wang B, Wang J, Shehan J, Sarma DP.(2008). Glomus tumor of the cheek. The Internet J ofDermatology 6 (2). Indexed by Google Scholar.

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Sarma DP, Wang B, Shehan J, Linder-Stephenson L (2007). Concurrent Merkel cell carcinoma and Bowen'sdisease of the thigh. The Internet J Dermatol 5(2). Indexed by Google Scholar.

The Internet Journal of Dermatology ISSN: 1531-3018

Concurrent Merkel cell carcinoma andBowen's disease of the thigh

Deba P. Sarma M.D. Department of Pathology, Creighton University Medical Center Omaha, NebraskaUSA

Bo Wang M.D. Department of Pathology, Creighton University Medical Center Omaha, Nebraska USA

James Shehan M.D. Department of Dermatology, Creighton University Medical Center Omaha,Nebraska USA

Lisa Linder-Stephenson M.A. Department of Pathology, Creighton University Medical Center Omaha,Nebraska USA

Citation: D.P. Sarma, B. Wang, J. Shehan, L. Linder-Stephenson: Concurrent Merkel cell carcinoma

and Bowen's disease of the thigh. The Internet Journal of Dermatology . 2007 Volume 5 Number 2

Keywords: Merkel cell carcinoma, Bowen's disease, concurrent tumor, collision tumor,neuroendocrine tumor of the skin

Abstract

We report a case of a Merkel cell carcinoma of the dermis with a Bowen's disease of the overlyingepidermis arising in the left thigh in an 88-year-old Caucasian female. A brief review of the

literature is presented.



Introduction

Merkel cell carcinoma (MCC) is a rare cutaneous malignant neuroendocrine tumor usually occurring in the head andneck of the elderly Caucasian patients. The histogenesis of such a tumor has not been fully understood.Morphologically, it resembles a small cell carcinoma of the lung and should always be differentiated from othersmall round blue cell tumors. Merkel cell carcinoma is an aggressive cutaneous tumor with frequent recurrence anda high incidence of nodal and distant metastasis. Bowen's disease or in-situ squamous cell carcinoma is a relativelycommon lesion occurring both in the son-exposed and sun-protected skin. Chronic sun exposure with actinic

damage, exposure to arsenic, HPV infection, and internal malignancy has been associated with Bowen's disease.We present a case of concurrent MCC and Bowen's disease occurring at a sun-protected location and without anyknown risk factors.

Case Report

An 88-year-old Caucasian female presented a left thigh skin lesion (Figure 1), which was clinically suspected to be a

pyogenic granuloma. After a diagnosis of Merkel cell carcinoma was made from the biopsy, the entire lesion wascompletely excised with clear margins. Microscopically (Figures 2 and 3), the dermal lesion was a highly cellularand mitotically active malignant tumor composed of undifferentiated small round blue cells. The tumor exhibited asomewhat trabecular pattern with extensive necrosis. The neoplastic cells were medium in size with scant rims ofcytoplasm and hyperchromatic nuclei with a salt and pepper chromatin pattern without discernable nucleoli. Someof nuclei showed nuclear molding with smudgy chromatin. Numerous apoptotic bodies were also noted.Immunostains demonstrated that the tumor cells were positive for cytokeratin 20 with a characteristic paranucleardot-like pattern (Figure 4). The tumor cells were focally positive for synaptophysin and chromogranin but werenegative for cytokeratin 7 and cytokeratin 5/6. Ultrastructurally, the tumor cells showed relatively scant cytoplasm

with scattered membrane-bound electron dense core granules in the subplasmalemmal location. The clinicalfinding, histopathologic features, ultrastructural observation, and immunoprofile were consistent with primarycutaneous MCC rather than a metastatic small cell carcinoma from the lung.



Figure 1: Clinicalpresentation.



Figure 2: Low magnification shows Merkelcell carcinoma in the dermis.



Figure 3: High magnification of Merkel cellcarcinoma.



Figure 4: Immunostain by CK20 showscharacteristic paranuclear dot stain patternin Merkel cell carcinoma.



Figure 5: Epidermis shows Bowen'sdisease.



The overlying epidermis revealed a typical Bowen's disease with dysplastic keratinocytes occupying the entirethickness of the epidermis (Figure 5). On immunostaining, the epidermal neoplastic cells were positive forcytokeratin 7 and 5/6 and negative for cytokeratin 20, synaptophysin, and chromogranin indicating that the Bowen'sdisease was a separate and distinct lesion occurring concurrently with the dermal MCC.

The patient remains free of recurrent or metastatic tumor four years after the excision of the lesion.

Discussion

Both Merkel cell carcinoma (MCC) of the skin (also called primary neuroendocrine carcinoma of the skin) and Bowen'sdisease are seen mostly in the sun-exposed skin. The etiologic association of UV light, arsenic exposure and HPVinfection has been established for Bowen's disease, but the cause of MCC remains unknown. Several factors, suchas, white skin, older age, sun exposure, and immunosuppression appear to be related to the development of MCC.There are several case reports (1, 2, 3, 4) describing the occurrence of MCC and Bowen's disease among thepeople exposed to arsenic. In these cases, the exposure to arsenic appears to be the possible carcinogenic factorleading to the development of MCC and Bowen's disease. There was no evidence of arsenic exposure in ourpatient. Interestingly, MCC has both neuroendocrine features, such as, neurofilaments and neuroendocrine

granules and epithelial features, such as, cytokeratin in their cytoplasm. It has been speculated (2) that thebidirectional differentiation potential of MCC is reflected in the reports where MCC has been associated with otherskin carcinomas, such as, squamous cell carcinoma (5, 6) and eccrine carcinoma (6). The Bowen's disease of theepidermis in our case does not show any continuity with the MCC in the dermis. Therefore, our observationsupports that there are two distinct lesions in the same site in our patient may be merely co-incidental. Clinicalrecognition of such a case of concomitant MCC and Bowen's disease, especially occurring in an unexpected sun-protected location may be difficult.

Correspondence to

Bo Wang, MD Department of Pathology Creighton University Medical Center Omaha, NE 68131 E-mail:[email protected]



References

1. Tsuruta D, Hamada T, Mochida K, Nakagawa K, Kobayashi H, Ishii M. Merkel cell carcinoma, Bowen's disease andchronic occupational arsenic poisoning. Br J Dermatol. 1998 Aug; 139(2):291-4.

2. Ohnishi Y, Murakami S, Ohtsuka H, Miyauchi S, Shinmori H, Hashimoto K. Merkel cell carcinoma and multipleBowen's disease: incidental association or possible relationship to inorganic arsenic exposure? J Dermatol. 1997May; 24(5):310-6.

3. Okamoto O, Yoshiyama M, Takayasu S, Yokoyama S. Merkel cell carcinoma: report of three cases. J Dermatol. 1998Jan; 25(1):45-50.

4. Schenk P, Konrad K. Merkel cell carcinoma of the head and neck associated with Bowen's disease. Eur ArchOtorhinolaryngol. 1991; 248(8):436-41.

5. Iacocca MV, Abemethy JL, Stefanato CM, Allan AE, Bhawan J. Mixed Merkel cell carcinoma and squamous cellcarcinoma of the skin. 1998; J Am Acad Dermatol 39(5 Pt 2):882-7.

6. Gould E, Albores-Saavedra J, Dubner B, Smith W, Payne CM. Eccrine and squamous differentiation in Merkel cell

carcinoma. An immunohistochemical study. 1988; Am J Surg Pathol 12(10): 768-72.

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Deba P Sarma and James Shehan publication: Concurrent Merkel cell carcinoma and Bowen's disease of the thigh