Gastric Metastasis of Merkel Cell Carcinoma: Case Report

Abstract

Merkel cell carcinoma (MCC) of skin is a rare tumor with aggressive behavior. Local recurrences, regional lymph node and distant metastases of MCC are frequent but to date metastasis to upper gastrointestinal tract was reported only a few. We present a 75-yearold man who had primary MCC at his left thigh. The tumor was excised and the patient was directed to local radiotherapy. He had developed abdominal and thoracal subcutaneous metastatic nodules within 5 and 8 months after surgical excision, respectively. In the tenth month, a second operation had to be performed because of a perforated duodenal ulcer and incidentally a submucosal yellow-white nodule measuring 2 cm in diameter was found in the wall of gastric cardia and excised. This lesion was histologically identical to the primary tumor, hence, gastric metastasis of MCC. The patient rejected further therapy and died 17 months after the initial diagnosis. Differential diagnosis of MCC and distinction of gastrointestinal MCC metastasis from primary neuroendocrine tumors may be difficult. Clinical information and histopathological features along with the results of immunohistochemical stainings are very important in this distinction. Derinin Merkel hücreli karsinomu (MHK) agresif davranışlı, nadir bir tümördür. Merkel hücreli karsinomun lokal rekürrensleri, bölgesel lenf nodu ve uzak metastazları sık olmakla birlikte bildirilen üst gastrointestinal metastaz oldukça azdır. Yetmiş beş yaşındaki erkek hasta, sol bacağındaki MHK eksize edildikten sonra lokal radyoterapiye yönlendirilmiştir. Tümör eksizyonundan sonraki 5. ayda karında, 8. ayda toraks duvarında subkutan metastatik nodüller gelişmiştir. İlk operasyondan 10 ay sonra perfore duodenal ülser nedeniyle yapılan ikinci operasyon sırasında rastlantısal olarak midede, kardia duvarında farkedilen 2 cm çaplı, sarı-beyaz renkli, submukozal nodül eksize edilmiştir. Bu lezyon MHK'un mide metastazı tanısı almıştır. Hasta daha ileri bir tedaviyi reddetmiş ve başlangıçtaki tanıdan 17 ay sonra yaşamını yitirmiştir. MHK'un ayırıcı tanısı ile gastrointestinal MHK metastazlarının primer nöroendokrin tümörlerden ayrımı zorluk yaratabilir. Bu tür tanı sorunlarının çözümünde klinik bilgi, histopatolojik özellikler ve immunohistokimyasal çalışmalar oldukça önemlidir.

Keywords

• Merkel cell carcinoma; stomach; gastrointestinal; metastasis

Midede Merkel Hücreli Karsinom Metastazı: Olgu Sunumu

Öz

Derinin Merkel hücreli karsinomu (MHK) agresif davranışlı, nadir bir tümördür. Merkel hücreli karsinomun lokal rekürrensleri, bölgesel lenf nodu ve uzak metastazları sık olmakla birlikte bildirilen üst gastrointestinal metastaz oldukça azdır. Yetmiş beş yaşındaki erkek hasta, sol bacağındaki MHK eksize edildikten sonra lokal radyoterapiye yönlendirilmiştir. Tümör eksizyonundan sonraki 5. ayda karında, 8. ayda toraks duvarında subkutan metastatik nodüller gelişmiştir. İlk operasyondan 10 ay sonra perfore duodenal ülser nedeniyle yapılan ikinci operasyon sırasında rastlantısal olarak midede, kardia duvarında farkedilen 2 cm çaplı, sarı-beyaz renkli, submukozal nodül eksize edilmiştir. Bu lezyon MHK'un mide metastazı tanısı almıştır. Hasta daha ileri bir tedaviyi reddetmiş ve başlangıçtaki tanıdan 17 ay sonra yaşamını yitirmiştir. MHK'un ayırıcı tanısı ile gastrointestinal MHK metastazlarının primer nöroendokrin tümörlerden ayrımı zorluk yaratabilir. Bu tür tanı sorunlarının çözümünde klinik bilgi, histopatolojik özellikler ve immunohistokimyasal çalışmalar oldukça önemlidir.

Anahtar Kelimeler

• Merkel hücreli karsinom, mide, gastrointestinal, metastaz

Kaynakça

1. Brenn T, McKee PH. Tumors of the surface epithelium. In: McKee PH, Calonje E, Granter SR, editors. Pathology of the skin with clinical correlations. 3rd ed. Philadelphia: Elsevier Mosby; 2005. p. 1153-1238.
2. Kohler S, Kerl H. Merkel cell carcinoma. World Health Organization Classification of Tumours. In: LeBoit PE, Burg G, Weedon D, Sarasin A, editors. Pathology and genetics of skin tumours. Lyon: IARC Press; 2006. p. 272-3.
3. Woodworth B, Lacey JP, Amedee RG. Merkel cell carci- noma: an overview and case report. J La State Med Soc 2001;153:522-6.
4. Linjawi A, Jamison WB, Meterissian S. Merkel cell carci- noma: important aspects of diagnosis and management. Am Surg 2001;67:943-7.
5. Coit DG. Merkel cell carcinoma. Ann Surg Oncol 2001;8(9 Suppl):99S-102S.
6. Medina-Franco H, Urist MM, Fiveash J, Heslin MJ, Bland KI, Beenken SW. Multimodality treatment of Merkel cell carcinoma: case series and literature review of 1024 cases. Ann Surg Oncol 2001;8:204-8.
7. Tai PT, Yu E, Tonita J, Gilchrist J. Merkel cell carcinoma of the skin. J Cutan Med Surg 2000;4:186-95.
8. Snow SN, Larson PO, Hardy S, Bentz M, Madjar D, Landeck A, et al. Merkel cell carcinoma of the skin and mucosa: report of 12 cutaneous cases with 2 cases arising from the nasal mucosa. Dermatol Surg 2001;27:165-70.

9. Jensen K, Kohler S, Rouse RV. Cytokeratin staining in Merkel cell carcinoma: an immunohistochemical study of cytokeratins 5/6, 7, 17, and 20. Appl Immunohistochem Mol Morphol 2000;8:310-5.
10. Akhtar S, Oza KK, Wright J. Merkel cell carcinoma: report of 10 cases and review of the literature. J Am Acad Dermatol 2000;43:755-67.
11. Gollard R, Weber R, Kosty MP, Greenway HT, Massullo V, Humberson C. Merkel cell carcinoma: review of 22 cases with surgical, pathologic, and therapeutic considerations. Cancer 2000;88:1842-51.
12. Chang SF, Suh JW, Choi JH, Yoon GS, Huh J, Sung KJ, et al. Diagnostic pitfalls of Merkel cell carcinoma and dramatic response to chemotherapy. J Dermatol 1998;25:322-8.
13. Collins BT, Elmberger PG, Tani EM, Bjornhagen V, Ramos RR. Fine-needle aspiration of Merkel cell carcinoma of the skin with cytomorphology and immunocytochemical cor- relation. Diagn Cytopathol 1998;18:251-7.
14. Skelton HG, Smith KJ, Hitchcock CL, McCarthy WF, Lupton GP, Graham JH. Merkel cell carcinoma: analysis of clinical, histologic, and immunohistologic features of 132 cases with relation to survival. J Am Acad Dermatol 1997;37:734-9.
15. An KP, Ratner D. Merkel cell carcinoma in the setting of HIV infection. J Am Acad Dermatol 2001;45:309-12.
16. Hapcic K, Panchal J, Stewart J, Levine N. Giant Merkel cell carcinoma involving the upper extremity. Dermatol Surg 2001;27:493-4.
17. Li M, Liu C. Cytokeratin 20 confirms Merkel cell metas- tasis to stomach. Appl Immunohistochem Mol Morphol 2004;12:346-9.
18. Idowu MO, Contos M, Gill S, Powers C. Merkel cell carci- noma: a report of gastrointestinal metastasis and review of the literature. Arch Pathol Lab Med 2003;127:367-9.
19. Canales LI, Parker A, Kadakia S. Upper gastrointestinal bleeding from Merkel cell carcinoma. Am J Gastroenterol 1992;87:1464-6.
20. Hizawa K, Kurihara S, Nakamori M, Nakahara T, Matsumoto T, Iida M. An autopsy case of Merkel cell carci- noma presenting aggressive intraabdominal metastasis and duodenal obstruction. Nippon Shokakibyo Gakkai Zasshi 2007;104:1383-6.
21. Cubiella J, Salgado M, Riş M, García-Mata J, Sánchez E, Díez MS, et al. Gastric metastatis due to Merkel cell carci- noma: a rare cause of gastric bleeding. Rev Esp Enferm Dig 2004;96:150-1. [Abstract]
22. Krasagakis K, Almond-Roesler B, Zouboulis CC, Tebbe B, Wartenberg E, Wolff KD, et al. Merkel cell carcinoma: report of ten cases with emphasis on clinical course, treat- ment, and in vitro drug sensitivity. J Am Acad Dermatol 1997;36:727-32.
23. Naunton Morgan TC, Henderson RG. Small bowel metasta- ses from a Merkel cell tumor. Br J Radiol 1985;58:1212-3.
24. Olivero G, Franchello A, Pacchioni D, Enrichens F, Mao P, Benedetto G. A rare case of Merkel's tumor with intestinal metastases. Ann Ital Chir 1990;61:277-80. [Abstract]
25. Shalhub S, Clarke L, Morgan MB. Metastatic Merkel cell carcinoma masquerading as colon cancer. Gastrointest Endosc 2004;60:856-8.
26. Leech SN, Kolar AJ, Barrett PD, Sinclair SA, Leonard N. Merkel cell carcinoma can be distinguished from metastatic small cell carcinoma using antibodies to cytokeratin 20 and thyroid transcription factor 1. J Clin Pathol 2001;54:727-9.
27. Cheuk W, Kwan MY, Suster S, Chan JK. Immunostaining for thyroid transcription factor 1 and cytokeratin 20 aids the distinction of small cell carcinoma from Merkel cell carcinoma, but not pulmonary from extrapulmonary small cell carcinomas. Arch Pathol Lab Med 2001;125:228-31.
28. Kontochristopoulos GJ, Stavropoulos PG, Krasagakis K, Goerdt S, Zouboulis CC. Differentiation between Merkel cell carcinoma and malignant melanoma: An immunohis- tochemical study. Dermatology 2000;201:123-6.
29. Devoe K, Weidner N. Immunohistochemistry of small round-cell tumors. Semin Diagn Pathol 2000;17:216-24.
30. Nicholson SA, McDermott MB, Swanson PE, Wick MR. CD99 and cytokeratin-20 in small-cell and basaloid tumors of the skin. Appl Immunohistochem Mol Morphol 2000;8:37-41.
31. Scott MP, Helm KF. Cytokeratin 20: a marker for diagnosing Merkel cell carcinoma. Am J Dermatopathol 1999;21:16-20.
32. Schmidt U, Müller U, Metz KA, Leder LD. Cytokeratin and neurofilament protein staining in Merkel cell carcinoma of the small cell type and small cell carcinoma of the lung. Am J Dermatopathol 1998;20:346-51.
33. O'Connor WJ, Roenigk RK, Brodland DG. Merkel cell carcinoma. Comparison of Mohs micrographic surgery and wide excision in eighty-six patients. Dermatol Surg 1997;23:929-33.
34. Tai PT, Yu E, Winquist E, Hammond A, Stitt L, Tonita J, et al. Chemotherapy in neuroendocrine/Merkel cell carcinoma of the skin: case series and review of 204 cases. J Clin Oncol 2000;18:2493-9.
35. Fenig E, Brenner B, Katz A, Rakovsky E, Hana MB, Sulkes A. The role of radiation therapy and chemotherapy in the treatment of Merkel cell carcinoma. Cancer 1997;80:881-5.