Management Of Merkel Cell Carcinoma Research Articles

Merkel Cell Carcinoma: Important Aspects of Diagnosis and Management

Merkel cell carcinoma (MCC) is a highly aggressive primary neuroendocrine tumor. It is suggested in the literature that postoperative radiotherapy may decrease local recurrence and improve overall survival. The purpose of this retrospective review was to determine our experience and review the literature on this aggressive malignancy. Charts of ten patients with MCC seen between 1985 and 1997 were reviewed to obtain clinicopathological data. Eight patients were male with a mean age of 72 years (range 49-90). The head and neck was the most common site, affecting 50 per cent of patients. All patients had primary excisions with documented negative margins. Pathological size ranged from 10 to 40 mm. Initial pathological diagnosis was lymphoma in three cases requiring immunohistochemistry for cytokeratin and neuron-specific enolase for definitive diagnosis. Lymphatic invasion was noted in three patients but only one of these patients had clinical lymph node involvement. The mean follow-up was 54 months (range 6-114) with an 80 per cent one-year survival and 30 per cent 2-year survival. Postoperative radiotherapy was administered to five patients. Of these three died with evidence of both local and distant recurrence. This small retrospective review highlights important points in the management of MCC including pathological diagnosis and benefits of adjuvant radiation therapy.

Merkel cell carcinoma in the setting of HIV infection

Merkel cell carcinoma has been found to have an increased incidence among immunosuppressed patients, specifically organ transplant recipients receiving immunosuppressive therapy. HIV similarly depresses the immune response of infected persons. We report a case of Merkel cell carcinoma (MCC) in an HIV-infected patient who died from liver metastases 2 years after his tumor was diagnosed. The purpose of this report is to describe the possible relationship between HIV and MCC and to emphasize the importance of early diagnosis and aggressive management of MCC. (J Am Acad Dermatol 2001;45:309-12.)

The role of postoperative radiotherapy in the management of merkel cell carcinoma

The role of postoperative radiotherapy in the management of merkel cell carcinoma

Unusual presentation of a Merkel cell carcinoma

Merkel cell carcinoma (MCC) is an uncommon, potentially lethal, cutaneous tumor that mainly occurs in sun-exposed skin of the head and neck area of the elderly. We report a case of MCC presenting as a 2-mm crusted erosion on the nose of an elderly patient, the smallest MCC reported thus far in the literature. The optimal management of MCC has not been clearly established. In view of its high local recurrence rate, predilection to metastasis, and significant mortality, aggressive treatment has been advocated. Identification of this tumor at such a small size posed a management dilemma because of lack of prospective treatment data involving biologic markers of prognostic significance for MCC. (J Am Acad Dermatol 2000;42:366-70.)

Multimodality management of merkel cell carcinoma: Ott MJ, Tanabe KK, Gadd MA. Arch Surg 134:388, 1999

Multimodality management of merkel cell carcinoma: Ott MJ, Tanabe KK, Gadd MA. Arch Surg 134:388, 1999

Multimodality management of Merkel cell carcinoma.

Merkel cell carcinoma is a rare dermal neuroendocrine carcinoma whose optimal treatment and prognostic factors are poorly defined. We hypothesize that high-risk patients with Merkel cell carcinoma are best treated with multimodality therapy. A retrospective review of all patients (N = 33) with Merkel cell carcinoma treated at the Massachusetts General Hospital from January 1, 1980, to August 24,1997. Median follow-up time was 37 months (range, 6-157 months). Adequate data for evaluation were available for 31 patients. Male to female distribution was 14 men and 17 women, with a median patient age of 68 years. Stage at presentation; factors associated with recurrence; and the effects of surgery, radiation therapy (XRT), and chemotherapy on recurrence, salvage, and survival rates. There were 12 extremity, 11 head and neck, and 8 truncal tumors. There were 22 isolated primary tumors, 8 with additional clinically positive lymph nodes, and 1 with distant disease. Therapy was local excision with or without XRT in 19 patients, local resection and lymphadenectomy with or without XRT in 8 patients, and XRT alone in 4 patients with head and neck tumors. Fifteen patients developed recurrences (7 local, 8 nodal, and 10 distant). Median time to recurrence was 8 months (range, 3-48 months). There were 7 tumor-related deaths, 6 of which were associated with truncal lesions (P<.001). No locoregional recurrences occurred in patients with margins of resection of 2 cm or greater or adequate XRT. A multivariate analysis selected truncal location (P = .005) and nodal disease (P = .05) as predictors of mortality. Remission was possible in 5 patients with locoregional and 2 patients with distant recurrences. Merkel cell carcinoma is an aggressive dermal cancer with frequent nodal metastases; truncal tumors have the worst prognosis. Locoregional recurrence correlates with inadequate margins and lack of XRT, but remission is possible with multimodality therapy.

Surgical management of Merkel cell carcinoma.

To characterize the natural history of Merkel cell carcinoma (MCC) and to analyze the influence of patient, tumor, and treatment-related variables on survival and recurrence. Approximately 425 cases of MCC have been described in the literature. This study represents the largest experience reported. A review was performed of patients who had been treated at Memorial Sloan-Kettering Cancer Center for MCC between 1969 and 1996. Follow-up data were available for 102 of the 109 (94%) patients identified. The overall 5-year disease-specific survival rate was 74%. The median follow-up was 35 months. For all patients, the only independent predictor of survival was the tumor stage at presentation. For patients with stage I disease, the tumor size at presentation was also an independent predictor of survival. Recurrence of disease occurred in 55 patients (55%), and the most common site of first recurrence was within the draining lymph nodes (n = 35). Elective lymph node dissection was the only parameter independently predictive of improved relapse-free survival. The overall disease-specific survival rate after recurrence was 62%. Predictors of improved disease-specific survival after recurrence included nodal as compared to local or distant recurrence, the ability to render the patient free of disease after recurrence, and a disease-free interval of >8 months. The prognosis for patients with MCC is favorable, and even after recurrence the majority of patients experience long-term survival. Incorporation of size into the staging system more accurately predicts survival in patients with stage I disease. Although elective lymph node dissection decreased the rate of recurrence, it was not associated with improved overall survival.

Modern concept in the management of Merkel cell carcinoma

Modern concept in the management of Merkel cell carcinoma

The natural history and management of Merkel cell carcinoma of the skin: A review of 22 patients treated at the Royal Marsden Hospital

Merkel cell carcinoma is a rare skin malignancy, which primarily affects the elderly. Currently, there is only limited data on the natural history of this condition and no consensus on its optimum management. We have reviewed the natural history and management of 22 patients with Merkel cell carcinoma, who were treated at the Royal Marsden Hospital between 1985 and 1994. The median age at diagnosis was 75 years (range 55–96), with the head and neck region being the most common site of disease (nine patients: 41%). Seventeen patients (77%) presented with skin disease, three (14%) with regional lymphadenopathy and two (9%) with metastatic disease. Of the Stage I patients, 41% developed local recurrence post-operatively at a median time to relapse of 12 months. Those with head and neck disease had the highest risk of local recurrence, which occurred in 62.5% of this group. Stage I patients also had a high risk of disease progression, with 53% developing regional lymphadenopathy or visceral metastases. The median survival for all disease stages was 47 months. The treatment of unresectable primary or recurrent disease with radiotherapy led to valuable long term control in four of nine patients treated. Six courses of chemotherapy were administered; one brief complete response was observed, occurring in a patient treated with cyclophosphamide, vincristine and doxorubicin. The data in this study confirms the predilection for the elderly and the aggressive nature of Merkel cell carcinoma, with only four of 17 Stage I patients remaining disease free. To clarify the role of adjuvant postoperative radiotherapy and to establish the appropriate use of chemotherapy in metastatic spread of this rare malignancy will require further studies with multicentre cooperation.

Characterization of cell lines established from Merkel-cell ("small-cell") carcinoma of the skin.

Six cell lines have been established from different biopsies of the rare small-cell skin cancer of neuro-endocrine origin known as Merkel-cell carcinoma (MCC). These were established from metastatic lesions and have now been in culture for periods varying from 1 to 3 years. All lines grow as suspension cultures and exhibit typical MCC cytological features of small round cells with little cytoplasm, round nuclei and tight junctions. There was variation in the number and size of nucleoli and dense-core granules. We present details of their immunohistochemistry and growth characteristics in culture. The latter were similar to small-cell lung-cancer (SCLC) lines and can be subdivided, by their appearance, into type I, type II and type III in the same way as SCLC lines. This classification may be of significance for the prognosis and management of MCC.

The treatment of advanced Merkel cell carcinoma. A multimodality chemotherapy and radiation therapy treatment approach.

The optimal management of Merkel cell carcinoma has not been clearly defined. To describe the treatment of eight patients who presented with Merkel cell carcinoma. Eight patients with advanced locoregional Merkel cell carcinoma were seen in our institute over a 7-year period. Four patients were successfully treated by induction chemotherapy after excisional biopsy, followed by radiotherapy. Three patients died from widespread metastases and one from neutropenic sepsis induced by chemotherapy. These cases demonstrate the potential lethality and aggressive nature of this rare skin tumor despite its chemosensitive and radioresponsive character. In all four patients who are alive and disease-free, chemotherapy was given first and radiotherapy was given as consolidation. We suggest that a multimodality treatment approach--chemotherapy followed by radiotherapy--is indicated in patients with advanced Merkel cell carcinoma.

Merkel cell carcinoma

Merkel cell carcinoma is a malignant neuroendocrine tumor with features of epithelial differentiation. Biologically aggressive, it may be difficult to diagnose and, particularly in its late stages, even more difficult to treat effectively. This article addresses what is known and what is still controversial about the histogenesis, diagnosis, and management of Merkel cell carcinoma and the structure and function of the Merkel cell from which it is believed to be derived. The incidence, clinical presentation and diagnosis, ultrastructure, immunocytochemistry, treatment, and prognosis of this tumor will be discussed.

The role of radiation therapy in the management of merkel cell carcinoma of the head and neck

The role of radiation therapy in the management of merkel cell carcinoma of the head and neck

Radiotherapy as an adjunct in the management of merkel cell carcinoma

Four patients with a diagnosis of Merkel cell carcinoma initially underwent surgery followed by radiotherapy. Recurrent disease prompted use of radiation in three cases. The three cases of recurrent disease illustrate the aggressiveness of Merkel cell carcinoma and also provide further documentation of the radiosensitivity of this tumor. Additionally, these cases suggest that surgery alone frequently is inadequate to achieve local control of disease.