Abstract
Background—Purpose
The risk of secondary malignancy development in patients with hairy cell leukemia has been evaluated in several studies with varying results. The aim of this study is to describe a case of synchronous occurrence of neuroendocrine colon carcinoma and hairy cell leukemia.
Methods
A 69-year-old man presented with rectal bleeding. Colonoscopy revealed a rectal tumor, whereas biopsy specimens revealed a poorly differentiated carcinoma. During the preoperative evaluation, pancytopenia was detected.
Results
At laparotomy, a mass was detected 16 cm from the anal verge and an anterior resection of the rectum was performed. Detailed histological and immunohistochemical analyses revealed a poorly differentiated neuroendocrine carcinoma of the rectum. Postoperative evaluation of pancytopenia revealed hairy cell leukemia. The patient was initially treated with chemotherapy for hairy cell leukemia followed by chemotherapy for neuroendocrine colon carcinoma. Survival was 44 months.
Conclusions
To our knowledge, synchronous occurrence of neuroendocrine colon carcinoma and hairy cell leukemia has not been previously reported in the literature. Given the rare incidence of both entities in the general population, it is highly unlikely that they occurred together by chance. Further research is needed to determine what would be the optimal management options of patients with simultaneous hairy cell leukemia and a neuroendocrine colon cancer.
Similar content being viewed by others
References
Bernstein L, Newton P, Ross RK. Epidemiology of hairy cell leukemia in Los Angeles County. Cancer Res. 1990;50:3605–9.
Staines A, Cartwright RA. Hairy cell leukaemia: descriptive epidemiology and a case-control study. Br J Haematol. 1993;85:714–7.
Hisada M, Chen BE, Jaffe ES, Travis LB. Second cancer incidence and cause-specific mortality among 3104 patients with hairy cell leukemia: a population-based study. J Natl Cancer Inst. 2007;99:215–22.
Mey U, Strehl J, Gorschlüter M, Ziske C, Glasmacher A, Pralle H, et al. Advances in the treatment of hairy-cell leukaemia. Lancet Oncol. 2003;4:86–94.
Au WY, Klasa RJ, Gallagher R, Le N, Gascoyne RD, Connors JM. Second malignancies in patients with hairy cell leukemia in BRITISH COLUMBIA: a 20-year experience. Blood. 1998;92:1160–4.
Jacobs RH, Vokes EE, Golomb HM. Second malignancies in hairy cell leukemia. Cancer. 1985;56:1462–7.
Kurzrock R, Strom SS, Estey E, O'Brien S, Keating MJ, Jiang H, et al. Second cancer risk in hairy cell leukemia: analysis of 350 patients. J Clin Oncol. 1997;15:1803–10.
Federico M, Zinzani PL, Frassoldati A, Vinceti M, Modè A, Annino L, et al. Risk of second cancer in patients with hairy cell leukemia: long-term follow-up. J Clin Oncol. 2002;20:638–46.
Kampmeier P, Spielberger R, Dickstein J, Mick R, Golomb H, Vardiman JW. Increased incidence of second neoplasms in patients treated with interferon alpha 2b for hairy cell leukemia: a clinicopathologic assessment. Blood. 1994;83:2931–8.
Pawson R, A'Hern R, Catovsky D. Second malignancy in hairy cell leukaemia: no evidence of increased incidence after treatment with interferon alpha. Leuk Lymphoma. 1996;22:103–6.
Flinn IW, Kopecky KJ, Foucar MK, Head D, Bennett JM, Hutchison R, et al. Long-term follow-up of remission duration, mortality, and second malignancies in hairy cell leukemia patients treated with pentostatin. Blood. 2000;96:2981–6.
Hoffman MA. Clinical presentations and complications of hairy cell leukemia. Hematol Oncol Clin North Am. 2006;20:1065–73.
Troussard X, Henry-Amar M, Flandrin G. Second cancer risk after interferon therapy? Blood. 1994;84:3242–4.
Lawlor E, O'Briain DS, Finn T, Ward R, Rogers FM, O'Brien AA, et al. The simultaneous presentation of peripheral T-cell lymphoma and hairy cell leukemia. Cancer. 1987;60:1537–44.
Lynch SA, Brugge JS, Fromowitz F, Glantz L, Wang P, Caruso R, et al. Increased expression of the src proto-oncogene in hairy cell leukemia and a subgroup of B-cell lymphomas. Leukemia. 1993;7:1416–22.
Ballen KK, Canoso R, Seiler M, Neish A, Bauer KA. Neuroendocrine carcinoma in a patient with hairy cell leukemia: a case report. Med Pediatr Oncol. 1992;20:349–51.
Schlette E, Medeiros LJ, Beran M, Bueso-Ramos CE. Indolent neuroendocrine tumor involving the bone marrow. A case report with a 9-year follow-up. Arch Pathol Lab Med. 2003;127:488–91.
Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13, 715 carcinoid tumors. Cancer. 2003;97:934–59.
Bernick PE, Klimstra DS, Shia J, Minsky B, Saltz L, Shi W, et al. Neuroendocrine carcinomas of the colon and rectum. Dis Colon Rectum. 2004;47:163–9.
Saclarides TJ, Szeluga D, Staren ED. Neuroendocrine cancers of the colon and rectum. Results of a ten-year experience. Dis Colon Rectum. 1994;37:635–42.
Jung SH, Kim HC, Yu CS, Chang HM, Ryu MH, Lee JL, et al. Clinicopathologic characteristics of colorectal neuroendocrine tumor. Korean J Gastroenterol. 2006;48:97–103. Article in Korean, with English Abstract.
Benz R, Siciliano RD, Stussi G, Fehr J. Long-term follow-up of interferon-alpha induction and low-dose maintenance therapy in hairy cell leukemia. Eur J Haematol. 2009;82:194–200.
Conflict of interest
The authors declare that they have no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Salemis, N.S., Pinialidis, D., Tsiambas, E. et al. Synchronous Occurrence of Neuroendocrine Colon Carcinoma and Hairy Cell Leukemia. J Gastrointest Canc 42, 131–136 (2011). https://doi.org/10.1007/s12029-010-9167-7
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12029-010-9167-7