Merkel cell carcinoma arising after therapeutic immunosuppression

Gooptu, C.; Woollons, A.; Ross, J.S.; Price, M.L.; Wojnarowska, Fenella; Morris, Peter J.; Wall, Sarah; Bunker, C.B.

doi:10.1111/j.1365-2133.1997.tb03802.x

Cited by 96 publications

(47 citation statements)

References 16 publications

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“…Merkel cell carcinoma is a rare neuroendocrine malignancy (APUDoma) of the skin, generally occurring in elderly patients, [7] the mean age at presentation being about 75 years [17]. Merkel cell carcinoma is principally a disease of the Caucasian race [12] with an incidence of 0.44 per 100,000 [8] and is abnormally high (8%) among immunosuppressed patients [2,6]. An analysis of the Surveillance, Epidemiology and End Results (SEER) database conducted by Hodgson (2005) reported that the incidence of MCC has increased threefold between 1986 and 2001 [8].…”

Section: Discussionmentioning

confidence: 99%

Spinal extra-dural metastasis from Merkel cell carcinoma: a rare cause of paraplegia

Kamath

Venkateswaran²,

Shetty

et al. 2007

Eur Spine J

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We report a rare case of Merkel cell carcinoma with extra-dural spinal metastasis causing paraplegia. There are only four reported cases in literature. A 57-year-old lady presented with a breast lump, multiple truncal skin swellings, low back pain and rapidly progressive paraplegia. MRI showed multiple epidural soft tissue masses causing neural compression. A biopsy from the truncal skin lesion was diagnosed as Merkel cell carcinoma (MCC). Posterior decompression and tumor debulking at all three sites of neural compression was performed. Histopathology of the epidural tumor was consistent with MCC and the diagnosis was confirmed by immuno-histochemistry staining for cytokeratin-20. She was started on chemotherapy and radiotherapy. One month after diagnosis she died due to extensive metastasis. The short term palliative response seen in our patient demonstrates the poor prognosis for patients with spinal metastasis.Keywords Merkel cell carcinoma Á Spinal metastasis Á Secondaries Á Paraplegia Case reportA 57-year-old diabetic lady presented with persistent low back pain and bilateral radiculopathy of 2 months duration followed by rapidly progressive weakness and numbness of the lower limbs. She also had loss of bowel and bladder control since 1 week. Multiple painless skin lumps over the chest and abdomen had appeared spontaneously a few months prior to presentation. There were no constitutional symptoms. General examination revealed multiple firm nontender shiny skin lumps approximately 3-4 cm in diameter on the anterior abdominal wall, chest and on the right breast. There were also firm and non-tender mobile lymph-nodes of 2-3-cm size in the right posterior triangle of the neck (Fig. 1a). There was no tenderness or pain on movements of the spine. She had a flaccid paraplegia, a left extensor plantar response and sensory hypoesthesia below the ninth thoracic dermatome bilaterally. The radiographs and CT scan done prior to admission were normal (Fig. 1b, c). An MRI showed epidural soft tissue masses causing neural compression at the eighth thoracic, fourth lumbar and first sacral levels. There were also with multiple soft tissue lesions in the paraspinal and retroperitoneal space--features suggestive of metastasis. There was no history to suggest immuno-compromised status and human immunodeficiency virus infection was eliminated by the appropriate laboratory tests.An FNAC of the lymph node, anterior abdominal wall mass and right breast was reported as a small cell tumor. An incisional biopsy from a skin lesion was reported as MCC. A laminectomy and tumor debulking at all three sites of compression was performed. Epidural tumor tissue sent for histopathology was reported as a high grade malignancy composed of epitheliod cells with scanty cytoplasm, markedly pleomorphic nulei having a ''salt and pepper'' chromatin (highly reminiscent of a neuro-endocrine origin). The tumor cells with numerous mitosis were predominantly disposed in a trabercular and cribriform pattern and also arranged around necrotic space...

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Section: Discussionmentioning

confidence: 99%

Spinal extra-dural metastasis from Merkel cell carcinoma: a rare cause of paraplegia

Kamath

Venkateswaran²,

Shetty

et al. 2007

Eur Spine J

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“…Second neoplasms are frequently associated to primary MCC, including skin carcinomas and non-cutaneous solid tumors as well as haematological diseases (26,27). This provides a strong argument for the performance of whole-body 18 FDG PET in the staging and re-staging of MCC.…”

Section: ------------------------------------------------------------mentioning

confidence: 99%

Clinical added-value of 18FDG PET in neuroendocrine-merkel cell carcinoma

et al. 2006

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Abstract. Merkel cell carcinoma (MCC) is a rare and highly malignant skin cancer with neuroendocrine differentiation. We studied the potential value of 18 FDG PET in the management of MCC. Eleven patients with MCC were examined by 18 FDG PET and PET-CT for staging purpose (n=4) or for detection of recurrence (n=7). Qualitative and quantitative interpretation of PET studies was performed routinely. 18 FDG PET observations were compared to clinical and radiological findings. In 6 patients, PET findings were also compared to histology. In 7 patients, the 18 FDG tumor uptake was compared to the MCC proliferative activity expressed by the Ki-67 index. 18 FDG PET was contributive in 10/11 MCC patients. In 7 patients, 18 FDG PET detected focal lesions or a disseminated stage of the disease including dermal, nodal and visceral metastases. In 3 patients, a normal 18 FDG PET confirmed complete remission of disease. Most MCC patients exhibited highly 18 FDG-avid sites suggestive of increased glucose metabolism. This imaging pattern was related to a high proliferative activity (Ki-67 index >50%). In 1 patient with a weakly proliferative nodal MCC (Ki-67<10%), a false negative result was yielded by metabolic imaging. In 4/11 patients, 18 FDG PET revealed an unsuspected second neoplasm in addition to MCC. It is concluded that whole-body 18 FDG PET may be useful in the management of MCC patients. However, a normal 18 FDG PET aspect cannot rule out MCC with low proliferative activity.

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“…In addition, most MCCs express cyto keratin 20 (KRT20; CK20), which distinguishes it from other neuroendocrine tumors (3,4). Risk factors for developing MCC include advanced age, prolonged lifelong UV exposure, and an immunocompromised state from solid organ transplantation or HIV1 infection (5)(6)(7). In addition, there is an increased incidence of MCC in patients with chronic lymphocytic leukemia (CLL) and other hematologic proliferative disorders (8,9).…”

Section: Introductionmentioning

confidence: 99%