1/265. Diagnostic relevance of chromosomal in-situ hybridization in Merkel cell carcinoma: targeted interphase cytogenetic tumour analyses. AIMS: To resolve the conflicting diagnoses of five pathologists (which included well-differentiated neuroendocrine carcinoma, malignant carcinoid, undifferentiated small-cell carcinoma, primitive neuroectodermal tumour, metastases of small-cell lung carcinoma (SCLC) and Merkel cell carcinoma (MCC)), and tumour-free lungs after necropsy, we investigated an alarmingly metastasizing MCC in a 32-year-old Caucasian man using chromosomal in-situ hybridization (CISH). Differences in incidence and course in males and females also prompted targeted analyses for chromosomes X and Y. The lesion was also analysed for p53 gene mutations. methods AND RESULTS: paraffin sections of the thorax, buccal lymph nodes and scalp tumours were stained with haematoxylin and eosin. immunohistochemistry was performed with antibodies against pancytokeratin, keratin 20, neuron-specific enolase (NSE), chromogranin, neurofilaments and vimentin, among others. Sections (5-6 microm) of the tumours were analysed with alpha-satellite probes for chromosomes 1, 6, 7, 11, 12, 17, 18, X and Y using CrSH; and exons 5-9 of the p53 gene were examined by polymerase chain reaction and single strand conformation polymorphism (PCR-SSCP) methods. Although positive for pancytokeratin, keratin 20, chromogranin, NSE, synaptophysin and vimentin, the similarity in antigen profiles expressed by SCLC and MCC prevented a definitive tumour diagnosis. Chromosomal in-situ hybridization, however, revealed trisomies 1 and 11, two frequent aberrations in MCC, and trisomy 18. Moreover, 71% of the tumour cells had two to three copies of X, whereas 98% of the cell nuclei in the hair follicles and normal epidermis (purported Merkel cell origins) displayed one x chromosome. No mutations were detected in the five exons of the p53 gene examined. CONCLUSIONS: Had CISH been performed earlier, treatment may have been tailored specifically to suit MCC, since MCC and SCLC have different therapeutic strategies. Finally, chromosome X may be of prognostic relevance in MCC, which apparently predominates in females and yet shows poorer prognosis in males, and hence be worthy of further investigation. ( info) |
2/265. brain metastasis of Merkel cell carcinoma. Case report and review of the literature. Merkel cell carcinoma (MCC) is a rare primary cutaneous neuroendocrine tumor that is locally aggressive and has potential for metastatic spread. However, brain metastases are rare, and therapy for such tumors has never reported. The authors present a 48-year-old woman with MCC of the left elbow and a right cerebellar metastasis. After the right cerebellar mass was totally resected, radiation treatment and chemotherapy were performed. Eight cases of brain metastasis have been reported in the literature, but only 5 have been presented in sufficient detail for analysis. Therapy for brain metastases has always been palliative whole-brain irradiation and chemotherapy except for our patient, who underwent total removal of the tumor and survived for 11 months without neurological deficit. Except in the case of 1 with a particularly radiosensitive MCC, the patients with brain metastases died within 9 months after detection of the brain lesions. If possible, aggressive excision of brain metastases as well as of the primary lesion should be done. ( info) |
3/265. Merkel cell carcinoma: an aggressive neoplasm. This article presents a rare case of Merkel cell carcinoma (MCC) of the skin. This tumor is also known as trabecular carcinoma or cutaneous small cell undifferentiated carcinoma (CSCUC). This patient had a palpable non-tender mass in her right upper thigh and a large palpable lymph node in the right inguinal area. Microscopic examination revealed Merkel cell carcinoma (MCC) in the skin of the upper thigh as well as in the inguinal lymph node. Immunoperoxidase stains confirmed the diagnosis. Due to its aggressiveness and very unfavorable prognosis, prompt diagnosis and adequate treatment are essential. Wide local excision with adequate margins followed by radiation therapy is the treatment of choice. ( info) |
| Merkel cell carcinoma (MCC) is a rare cutaneous malignancy characterized by an aggressive clinical behavior with high rates of locoregional and systemic recurrence. Regional disease and distant metastases are associated with poor prognosis. Despite a predisposition of MCC to spread via the lymphatics, prophylactic lymph node dissection in the absence of clinically apparent lymph node involvement is controversial. The value of lymphoscintigraphy in cutaneous melanoma is established in lesions with ambiguous lymphatic drainage patterns. When used with sentinel lymph node biopsy (SLNB), it can identify subjects with occult regional node metastasis. The authors present 2 patients with MCC who underwent regional node staging with lymphoscintigraphy-directed SLNB. Both patients had sentinel nodes that were positive for metastatic disease. In patients with MCC, minimally invasive regional node staging SLNB may be useful in limiting the sequelae of routine lymphadenectomies. Whether early identification and treatment of patients with occult regional node disease can influence survival in MCC is not known. ( info) |
5/265. Ampullary somatostatinoma in a patient with Merkel cell carcinoma. A 59-yr-old white man with Merkel cell carcinoma of his right leg status post extensive skin resection and chemotherapy had dilated hepatic and common bile ducts on a routine follow-up abdominal CT scan. A 1.9-cm ampullary mass was appreciated on endoscopy. histology showed psammoma bodies and positive immunoperoxidase staining consistent with a somatostatinoma. Merkel cell tumors and somatostatinomas are extremely rare neuroendocrine tumors derived from neural crest cells. Associations have been found between somatostatinomas and other islet cell tumors with multiple endocrine neoplasia syndromes, but no reported association has been published between islet cell tumors and Merkel cell tumors. This patient represents the first documented case of Merkel cell carcinoma and somatostatinoma in a single patient. Such an occurrence may represent a previously undescribed neuroendocrine tumor syndrome, and this possibility should be considered when either tumor is diagnosed. ( info) |
6/265. Sentinel-node guided lymph-node dissection for merkel cell carcinoma. Merkel cell carcinoma is an aggressive neuroendocrine skin tumour. Treatment is still debatable. Merkel cell carcinoma resembles malignant melanoma in its cutaneous presentation and its embryonic origin; both have unpredictable biological behaviour, early regional lymph node involvement, early distant metastases and a high recurrence rate. In light of these common features, we used pre-operative lymphoscintigraphy, intraoperative lymph-node mapping and sentinel-node biopsy-a well-described technique for the treatment of melanoma-in a 60-year-old man with Merkel cell carcinoma in the right buttock. Following frozen section identification of a metastatic first-order sentinel node, radical right groin dissection was performed. All the other lymph nodes in this basin proved to be disease-free, including the second-order sentinel node and Cloquet node. The patient is now being treated with adjuvant chemotherapy and radiotherapy. This case shows that sentinel-node guided dissection is applicable to Merkel cell carcinoma. ( info) |
7/265. Merkel cell carcinoma occurring in renal transplant patients. Merkel cell carcinoma is a rare, highly aggressive tumor that usually affects the head and neck of elderly patients. We describe 3 cases of this high-grade, malignant tumor occurring in the setting of renal transplantation. ( info) |
8/265. Merkel cell carcinoma of the lower lip: a case report and histopathologic study. Merkel cell carcinoma of the lower lip is a rare clinical entity. It is generally thought to be more aggressive than the more common skin cancers of the lips and oral cavity. The literature reveals that delayed diagnoses and poor survival are common among patients with these malignancies. Recommended treatment is surgical excision with wide margins and treatment of cervical metastases with either surgery or radiotherapy or both. Unlike other malignant neoplasms, the occurrence of Merkel cell carcinoma in a relatively older population makes comorbid conditions a key factor in treatment planning. ( info) |
| BACKGROUND: Merkel cell carcinoma (MCC) is an aggressive cutaneous malignancy with a high incidence of occult nodal metastases. MCC is believed to be similar in natural history to thick or ulcerated melanomas in its propensity for locoregional recurrence and early lymph node metastasis. Studies have shown that nodal status is statistically correlated to survival in MCC. Radiolocalization and superselective lymph node biopsy is a recent technique that has been proven to be of great value in evaluating the status of occult lymph node disease in malignant melanoma and breast cancer patients. OBJECTIVE: In previously untreated patients, an orderly progression of metastases is observed for both cutaneous carcinomas and malignant melanomas and is anticipated for MCC. methods/RESULTS. We present two patients with MCC of the head and neck who underwent simultaneous Mohs micrographic surgery and sentinel lymph node biopsy with intraoperative radiolocalization. CONCLUSION: sentinel lymph node biopsy and intraoperative lymphoscintigraphy may prove to be a useful technique in evaluating occult nodal involvement and in limiting the potentially unnecessary morbidity of more comprehensive lymph node dissections in MCC patients who do not yet have metastatic involvement. ( info) |
10/265. 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. ( info) |