Chronic lymphocytic leukemia, lymphoma, merkel cell carcinoma

Increased incidence of chronic lymphocytic leukaemia and lymphomas in patients with Merkel cell carcinoma - a population based study of 335 cases with neuroendocrine skin tumour.

Mar 2012

Tadmor T, Liphshitz I, Aviv A, Landgren O, Barchana M, Polliack A.

Source

Haematology, Oncology Unit, Bnai-Zion Medical Centre, Haifa, Israel.

Abstract

Merkel cell carcinoma (MCC) is a rare aggressive skin tumour that appears to be associated with a large number of other tumours. We collected all reported cases in Israel and estimated its association with other tumours, including haematological malignancies. The population based Israel Cancer Registry identified 335 patients with MCC diagnosed between1989 and 2010. Ninety-seven percent were in the Jewish population; median age at diagnosis for Jewish patients was 73·4 and 55·6 years for the Arab population. Other associated malignancies were encountered in 92 patients (27·4%) with MCC (90 Jews, two Arabs). Of the Jewish cases, 66 presented with an associated malignancy before, and 24 after, the diagnosis of MCC. Solid tumours were not significantly increased among patients with MCC. Thirty-one of these associated cancers (34·4%) were haemato-oncological malignancies, 24 were detected before and seven after the diagnosis of MCC. The standardized incidence ratio (SIR) for haematological malignancy was 3·67 for males and 3·62 for females, and the most frequent haemato-oncological neoplasias recorded were chronic lymphocytic leukaemia (45%) and lymphomas (29%). Although MCC is rare, clinicians should be aware of the possible association with B-cell lymphoproliferative disorders when evaluating patients with neuroendocrine skin tumours.

Wiley OnLine

Significant overexpression of the Merkel cell polyomavirus (MCPyV) large T antigen in Merkel cell carcinoma.

Feb 2012

Erovic BM, Al Habeeb A, Harris L, Goldstein DP, Ghazarian D, Irish JC.

Source

Department of Otolaryngology-Head and Neck Surgery/Surgical Oncology, University Health Network, Princess Margaret Hospital, Toronto, Ontario, Canada.

Abstract

BACKGROUND:

The purpose of this study was to determine the expression pattern of the Merkel cell polyomavirus (MCPyV) large T-protein antigen in patients with Merkel cell carcinoma.

METHODS:

A tissue microarray (TMA) containing 30 specimens was constructed and stained for the MCPyV large T protein. Immunohistochemical expression was determined semiquantitively and was compared to patients' outcome.

RESULTS:

Nuclear expression of MCPyV large T protein was detected in 29 of 30 specimens (97%). In particular, 60% to 100%, 30% to 60%, and 10% to 30% of tumor cells were positive in 27 specimens (90%), 1 (3%), and 1 (3%), respectively. There was no difference in positivity between primary and metastatic lesions. Clinical data could not be correlated to MCPyV large T-protein expression.

CONCLUSION:

MCPyV large T protein was significantly overexpressed in 97% of all specimens. Although we could not demonstrate a predictive effect, MCPyV large T protein may represent a molecular marker with utility in pathological diagnosis as well as a potential new therapeutic target in patients with Merkel cell carcinoma. .

Wiley Online

Merkel Cell Carcinoma Concurrent with Bowen's Disease.

Feb 2012

Park HC, Kang HS, Park KT, Oh YH, Yu HJ, Kim JS.

Source

Department of Dermatology, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri, Korea.

Abstract

Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous malignancy of the elderly and immunocompromised patients. It is occasionally found coexisting with other diseases, such as squamous cell carcinoma, basal cell carcinoma, actinic keratosis, miscellaneous adnexal tumors, and rarely Bowen disease. A 75-year-old woman presented with a 6-month history of an irregularly shaped erythematous patch on the left mandibular angle. Three months later, a 1.5×1.0 cm sized painless and rapidly growing erythematous nodule developed on the patch. Microscopically, the patch lesion was consistent with that of Bowen disease. The nodular lesion showed a number of small uniform hyperchromatic cells with scanty cytoplasm. It showed dense small-cell like nodular infiltration in the dermis. Immunohistochemical staining for cytokeratin 20 showed a positive result with a dot-like perinuclear pattern. Additionally, the result for thyroid transcription factor-1 was negative, which is positive in small cell neuroendocrine carcinoma. From these findings, we diagnosed this lesion as MCC concurrent with Bowen disease.

Annals of Dermatology

Merkel cell carcinoma: case report.

Dec 2011

Richardson B, Avshalumova L, Miller R.

Source

University Hospitals Case Medical Center, Cleveland, Ohio, USA. blakely.richardson@uhhospitals.org

Abstract

Merkel cell carcinoma (MCC), also termed cutaneous small cell carcinoma or trabecular carcinoma, is a rare tumor that most often presents as a solitary nodule on the head, neck, or extremities of older adults. It is an aggressive tumor that usually is fatal due to rapid metastasis. Involvement of lymph nodes at presentation can be used to predict survival. Because MCC is sensitive to radiation, it can be used as an adjunct to surgery. We report a case of MCC to alert clinicians of this potentially fatal tumor because early diagnosis and proper treatment may improve patient survival rates.

PubMed

Merkel cell carcinoma.

Swann MH, Yoon J.

Source

Department of Dermatology, University of Missouri-Columbia, Columbia, MO 65212, USA. swann.mike@gmail.com

Abstract

Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous cancer that predominately affects elderly Caucasians with fair skin and has a propensity for local recurrence and regional lymph node metastases. A variety of terms have been used to describe this tumor, including trabecular cell carcinoma, neuroendocrine or primary small cell carcinoma of the skin, and anaplastic cancer of the skin. Although the skin lesion is most commonly found on sun-exposed areas of the head and neck or extremities, it can occur on the trunk, genitalia, and perianal region. The median age is 69 years, but it may occur earlier and more frequently in immunosuppressed patients. Patients with MCC frequently present with a nonspecific erythematous or violaceous firm nodule or small plaque that may be surrounded by small satellite tumors. MCC usually arises in the dermis and extends into the subcutis. It may be difficult to accurately diagnose MCC by light microscopy alone and ancillary techniques, including electron microscopy and immunohistochemistry, may be necessary to make a definitive diagnosis. Management of MCC is dependent on stage of the disease and is hampered by its rarity and lack of randomized trials. Nonetheless, for localized disease most guidelines include wide local excision of the primary tumor either alone or with radiation therapy. Sentinel lymph node biopsy can be helpful in staging and prognosis, but its benefit in survival remains to be seen. Systemic chemotherapy, akin to regimens for small cell carcinoma of the lung, may be considered as an adjuvant following surgery or to treat locoregional or distant disease. The prognosis of MCC is variable. Some patients with localized disease have an indolent course and are well controlled with local excision alone. On the other hand, many tumors are aggressive and have a tendency for locoregional recurrence and distant metastases. Such patients have a grim prognosis with a median survival of 9 months. Successful outcomes are most often seen in patients with early diagnosis and complete excision.

Elsevier