Merkel cell carcinoma of the auricle

Merkel cell carcinoma is a rare malignant tumor of the skin. The face and extremities are the most common locations. We present a case of Merkel cell carcinoma of the auricle in a 93-year-old man subsequently treated with surgery and radiotherapy. The clinical features, prognosis, and treatment of the Merkel cell carcinoma are discussed.     

Merkel cell carcinoma of the head and neck associated with Bowen's disease

Summary The Merkel cell carcinoma occurs primarily in the skin of the head and neck, and develops in the dermis with a trabecular growth pattern. Immunohistochemistry reveals positive staining for neuron-specific enolase, neurofilaments, cytokeratin and chromogranin A. Electron microscopically, the tumor cells contain dense-core granules, spinous cytoplasmic processes, desmosomes, zonulae adherentes and paranuclear filament aggregates besides frequent mitoses, focal necroses and lymphocyte and plasma cell infiltrates. The Merkel cell carcinoma is often co-existent with other malignancies such as squamous cell carcinoma or, as in the present study, with Bowen's disease. The definite diagnosis of the Merkel cell carcinoma can be effected only by electron microscopic examination of the tumor.Presented in part at the 62nd Annual Meeting of the German Society for Otorhinolaryngology, Head and Neck Surgery, May 14, 1991, Aachen, FRGDedicated to Prof. K. Burian on the occasion of his 70th birthday     

Oropharyngeal metastasis of a Merkel cell carcinoma of the skin

Merkel cell carcinoma (MCC) is an aggressive neuroendocrine tumor of the skin characterized by frequent local and regional recurrence, a high incidence of distant metastases and therefore a high mortality. Here, we report a case of an oropharyngeal metastasis of a Merkel cell carcinoma of the skin localized at the left base of the tongue causing dysphagia and the sensation of globus pharyngeus. The unusual metastatic site is presented, and diagnosis and treatment are discussed.     

The Merkel cell carcinoma

The Merkel cell carcinoma is a rare tumor of the subdermal tissue. Its origin are Merkel cells or the subcutaneous pluripotential stem cells. The etiology of the tumor is unknown, however, exposition to UV radiation and immunosuppression are relevant predisposing factors. Clinically, a subcutaneous rapidly growing tumor with or without enlarged lymph nodes is seen. Beside excisional biopsy and various imaging modalities, somatostatin-receptor-scintigraphy plays an important role in diagnostic. Surgical resection of the tumor combined with neck dissection followed by radiation therapy is the current therapy of choice. Different chemotherapy protocols are available for patients with distant metastasis.     

Merkel cell carcinoma of unknown primary site

Merkel cell carcinoma is an uncommon and aggressive primary neuroendocrine skin malignancy which mostly affects the extremities and the head and neck region of elderly patients. Merkel cell carcinoma occurs with increased frequency in sun-exposed areas, in individuals exposed to arsenic and in immunosuppressed patients. Many patients with Merkel cell carcinoma present with other malignancies, mainly skin cancers. Characteristic features are frequent recurrences and regional and distant metastases. Mortality rates range from 20 to 65 per cent. The mainstay of treatment is surgery, with wide local excision, and adjuvant radiotherapy is usually administered. Merkel cell carcinoma of unknown primary site is rare, and the majority of the few cases described have not been from head and neck areas. We present a case of Merkel cell carcinoma of unknown primary site, with upper neck and distant metastases.     

Merkel cell carcinoma of the skin with leptomeningeal metastases

Merkel cell carcinoma is a rare skin tumor that is thought to arise from epithelial cells that have undergone neuroendocrine differentiation. It usually presents in older adults and has a slight male predominance. The most frequent site of occurrence is in the head and neck. It is an aggressive disease that has a high rate of local-regional and distant recurrence. Optimal treatment is controversial but generally consists of surgery and adjuvant radiotherapy. The role of chemotherapy is less defined. This report documents the first known case of Merkel cell carcinoma with perineural spread to the central nervous system with leptomeningeal dissemination. Whether this represents a more aggressive variant is unknown. Regardless, this pattern of spread is likely a rare event.     

Merkel cell-carcinoma in the parotid gland--review of literature and case report

Merkel cell carcinoma is a rare malignant primary tumor of the skin, which develop typically in the elderly in the sun exposed areas of the skin, most commonly in the head and neck region. This tumor is very aggressive with a high rate of regional and distant metastases, marked propensity for local et regional recurrence, and very poor survival. Skin lesions are typically red or purple and present as a solitary dome-shaped nodule or indurated plaque. The recognition of such tumor in not difficult, but in same cases, when the tumor infiltrate deeper organs and structures it may cause some problems. We describe a case of 86 years old women who was operated in our department with an initial diagnosis of parotid tumor infiltrating the skin of the preauricular region and the facial nerve. The histopatological and immunohistochemical examination of the tumor showed that it was Merkel cell carcinoma, which in fact developed in the skin and infiltrated parotid gland and facial nerve. After operation patient received 2 courses of palliative radiation. Until now during a 4,5 years follow up there was no recurrence of the tumor and the patient is well.     

Merkel cell carcinoma (endocrine carcinoma of the skin) of the head and neck

Merkel cell carcinoma, also known as endocrine carcinoma of the skin, is a recently recognized and particularly aggressive form of skin cancer that exhibits histologic features similar to those of endocrine malignant neoplasms arising from other tissues. Forty-one patients with Merkel cell carcinoma arising from the cutaneous surfaces of the head and neck were seen at the University of Texas M. D. Anderson Hospital, Houston, between 1966 and 1983. Regional lymph node metastasis occur early and frequently, with a 79% overall incidence observed during the course of the disease. Treatment should consist of a wide resection of the primary tumor. A regional lymphadenectomy, when feasible, is successful in controlling nodal metastases. Postoperative radiation is recommended as an important adjuvant.     

The application of sentinel node radiolocalization to solid tumors of the head and neck: a 10-year experience

OBJECTIVES/HYPOTHESIS: The goals of the research study were to develop an easily mastered, accurate, minimally invasive technique of sentinel node radiolocalization with biopsy (SNRLB) in the feline model; to compare it with blue-dye mapping techniques; and to test the applicability of sentinel node radiolocalization biopsy in three head and neck tumor types: N0 malignant melanoma, N0 Merkel cell carcinoma, and N0 squamous cell carcinoma. STUDY DESIGN: Prospective consecutive series studies were performed in the feline model and in three head and neck tumor types: N0 malignant melanoma (43 patients), N0 Merkel cell carcinoma (8 patients), and N0 squamous cell carcinoma (20 patients). METHODS: The technique of sentinel node radiolocalization with biopsy was analyzed in eight felines and compared with blue-dye mapping. Patterns of sentinel node gamma emissions were recorded. Localization success rates were determined for blue dye and sentinel node with radiolocalization biopsy. In the human studies, all patients had sentinel node radiolocalization biopsy performed in a similar manner. On the morning of surgery, each patient had sentinel node radiolocalization biopsy of the sentinel lymph node performed using an intradermal or peritumoral injection of technetium Tc 99m sulfur colloid. Sentinel nodes were localized on the skin surface using a handheld gamma detector. Gamma count measurements were obtained for the following: 1) the "hot" spot/node in vivo before incision, 2) the hot spot/node in vivo during dissection, 3) the hot spot/node ex vivo, 4) the lymphatic bed after hot spot/node removal, and 5) the background in the operating room. The first draining lymph node(s) was identified, and biopsy of the node was performed. The radioactive sentinel lymph node(s) was submitted separately for routine histopathological evaluation. Preoperative lymphoscintigrams were performed in patients with melanoma and patients with Merkel cell carcinoma. In patients with head and neck squamous cell carcinoma, the relationship between the sentinel node and the remaining lymphatic basin was studied and all patients received complete neck dissections. The accuracy of sentinel node radiolocalization with biopsy, the micrometastatic rate, the false-negative rate, and long-term recurrence rates were reported for each of the head and neck tumor types. In the melanoma study, the success of sentinel node localization was compared for sentinel node radiolocalization biopsy, blue-dye mapping, and lymphoscintigraphy. In the Merkel cell carcinoma study, localization rates were evaluated for sentinel node radiolocalization biopsy and lymphoscintigraphy. In the head and neck squamous cell carcinoma study, the localization rate of sentinel node radiolocalization biopsy and the predictive value of the sentinel node relative to the remaining lymphatic bed were determined. All results were analyzed statistically. RESULTS: Across the different head and neck tumor types studied, sentinel node radiolocalization biopsy had a success rate approaching 95%. Sentinel node radiolocalization biopsy was more successful than blue-dye mapping or lymphoscintigraphy at identifying the sentinel node, although all three techniques were complementary. There was no instance of a sentinel node-negative patient developing regional lymphatic recurrence. In the head and neck squamous cell carcinoma study, there was no instance in which the sentinel node was negative and the remaining lymphadenectomy specimen was positive. CONCLUSION: In head and neck tumors that spread via the lymphatics, it appears that sentinel node radiolocalization biopsy can be performed with a high success rate. This technique has a low false-negative rate and can be performed through a small incision. In head and neck squamous cell carcinoma, the histological appearance of the sentinel node does appear to reflect the regional nodal status of the patient.     

Parotid metastases of a neuroendocrine cutaneous carcinoma

A case of metastatic Merkel-cell carcinoma to lymph nodes on the left side of the neck nad left parotid is described. Neuroendocrine cutaneous carcinoma, also called Merkel cell carcinoma (MCC), is an uncommon primary skin tumor most often seen in elderly. Literature is reviewed and comment about more important features of these lesions, like differential diagnosis, prognosis and treatment.     

Intracranial spread of Merkel cell carcinoma through intact skull

We report an unusual case of Merkel cell carcinoma presenting as a frontal scalp mass with apparent invasion into underlying brain parenchyma through grossly intact calvaria. Despite wide local excision, craniectomy, intracranial tumor resection, and postoperative adjuvant irradiation, widespread systemic metastases resistant to chemotherapy developed, and the patient died 9 months after surgery. This case report confirms that Merkel cell carcinoma of the head and neck, already known to be an aggressive tumor, has the capacity for rapid intracranial extension. We propose that in this case, the mechanism of intracranial metastasis was via communicating veins rather than through bone destruction or systemic metastasis. Appropriate preoperative imaging should be carried out to define the extent of this tumor when it is adjacent to the skull. We found contrast-enhanced magnetic resonance imaging to be superior to computed tomography for defining soft tissue extent and marrow space involvement within underlying bone.     

Merkel cell carcinoma: An aggressive skin neoplasm

Merkel cell carcinoma (MCC) is an aggressive skin neoplasm of neuroendocrine origin. To clarify those factors important in improving survival, we retrospectively reviewed the charts of all patients with Merkel cell carcinoma treated at two tertiary referral centers. Eighty percent of the patients with stage I disease were initially treated with local therapy alone, while all of the patients with stage II disease were treated with local and regional therapy. The overall survival rate for all patients was 64%. Regardless of stage, patients treated with local excision alone had a 52% 5-year survival rate, while patients treated with local excision and lymph node dissection had an 87% survival rate. We conclude that the aggressive nature of this tumor warrants radical therapy.     

Merkel cell carcinoma

PURPOSE: To determine the natural history and treatment outcomes for patients with Merkel cell carcinoma. METHODS: Review of the literature. RESULTS: The probability of regional node involvement at presentation exceeds 50%; few patients present with distant metastases. Comprehensive treatment of the primary site and regional lymphatics with surgery or radiotherapy results in the highest likelihood of cure. The role of adjuvant chemotherapy remains investigational. CONCLUSION: The probability of regional dissemination at diagnosis is high. The optimal treatment is resection of the primary tumor and treatment of the regional lymphatics. Resection of all gross tumor should be accomplished followed by local-regional radiotherapy in most patients.     

Merkel cell carcinoma of the upper lid]

AIMS: Merkel cell carcinoma (MCC) of the skin is a rare form of cutaneous malignancy of neuroendocrine origin with a propensity to form cutaneous and lymphogenous metastases. The tumor, affecting predominately elderly patients, has a significantly higher incidence in female patients (80%) compared to male patients (20%). The mainstay of therapy is surgical resection with possible adjuvant radio- or chemotherapy. CASE REPORT: A case of a 78-year-old patient with a MCC of the upper lid without lymph node involvement or distant metastasis is described. The tumor was resected and the defect closed by a temporal skin flap performing a temporary cantholysis. The clinical follow-up revealed an undisturbed eye lid function without any clinical or radiological signs of a locoregional or distant recurrence.     

Merkel cell carcinoma of the nose

Despite an increasing number of reports Merkel cell carcinoma still is a rare neoplasm. We therefore present the imaging feature of the case of Merkel cell carcinoma involving the nasal region in 65-year woman with lymphoma malignum lymphoplasmocytoides and its rapid progression with fast metastasis to lymph nodes. Surgery and adjuvant radiotherapy, chemotherapy appeared to provide optimal local control.     

Merkel-cell tumor associated with a spinocelluar epithelioma. Apropos a case report

One case of Merkel cell carcinoma is reported in a 71 years-old woman. Lately treated by surgical excision (14 years after the occurrence of the tumour), this tumour was already associated with inguinal lymph node metastases. Its evolution was lethal within 3 months after local recurrence, cutaneous and visceral (lung, liver) metastases. This tumour, characteristic histologically and ultrastructurally (secretory granules), was accompanied by an infiltrative epidermoid carcinoma. This association and the presence of cytokeratin in tumoral Merkel cells are arguments for the common origin of keratinocytes and neuroendocrine Merkel cells from a common young epithelial cell.     

Merkel cell carcinoma of the head and neck: effect of surgical excision and radiation on recurrence and survival

BACKGROUND: Merkel cell carcinoma is a rare malignant neoplasm of the skin that most often arises in the head and neck region. Despite the innocuous appearance of the primary lesion, Merkel cell carcinoma often has an aggressive clinical course with frequent locoregional recurrences and distant metastases. We evaluated the association of the width of surgical margins and the use of postoperative radiation therapy with locoregional control and survival rates. METHODS: The medical records of 66 patients with head and neck Merkel cell carcinoma seen between 1945 and 1995 were retrospectively reviewed. The Fisher exact test was used to compare outcomes. Kaplan-Meier survival curves were constructed. RESULTS: Eighteen patients for whom there was adequate information were divided into the following groups according to the width of their surgical margins: smaller than 1 cm, 1 to 2 cm, and larger than 2 cm. No statistical difference in locoregional control or survival was found among these groups owing to the small patient population. In contrast, a comparison of the patients who did (n = 26) and did not (n = 34) receive postoperative radiation therapy revealed a significant difference in local (3 [12%] vs 15 [44%], respectively; P<.01) and regional (7 [27%] vs 29 [85%], respectively; P<.01) recurrence rates. There was, however, no significant difference in the disease-specific survival between these groups (P = .30). Distant disease developed in 36% of all patients regardless of therapy. CONCLUSIONS: Any effect of the width of surgical margins on outcome was not detectable in the small number of patients analyzed. The use of postoperative radiation therapy was associated with a significant improvement in locoregional control. There was no detectable influence of the type of initial therapy on the rates of distant metastases or on survival. Future therapeutic innovations should be directed toward controlling the development of distant metastases in patients with Merkel cell carcinoma.     

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.     

Merkel cell carcinoma of the head and neck

Merkel cell carcinoma is a rare cutaneous tumour that typically arises in the head and neck area of elderly patients. The tumour often follows an aggressive course with frequent local recurrences and (regional) metastases, especially when localized above the clavicles. Five patients with a Merkel cell carcinoma of the head and neck, treated in our institute since 1984, are presented, illustrating the need for radical initial treatment consisting of surgery and radiotherapy, and showing how rapidly progressive the disease can be. Four of the five patients were cured of disease by a combination of surgery and radiotherapy.     

An analysis of prognostic factors in cutaneous neuroendocrine carcinoma.

Since the first report of cutaneous neuroendocrine carcinoma (Merkel cell carcinoma) in 1972, knowledge of the biological behavior of this tumor has continued to increase. This study reviews three previously unreported cases in addition to reports on 429 patients collected from the literature. The presenting features of these 306 patients were analyzed for frequency and then were analyzed by the Cox proportional hazard model for the relationship of each to recurrence and outcome. This study confirms that cutaneous neuroendocrine carcinoma is an aggressive disease; that the most common cause of death in these patients is by distant metastasis; and that men have succumbed more frequently than women. As a result of this study, aggressive treatment of cutaneous neuroendocrine carcinoma is recommended, especially in the male patient.