Merkel cell carcinoma of the skin

Primary neuroendocrine carcinoma of the skin or Merkel cell carcinoma is an aggressive primary neoplasm. It is commonly seen in the elderly, on the head, neck and extremities, where it can mimic a benign or less malignant skin tumour. Pathological examination shows a generally dense growth of small dark cells, with immunohistochemical evidence of neuroendocrine differentiation. The microscopic appearance is very similar to metastatic oat cell carcinoma from the lung and this must be excluded by clinical means and appropriate imaging studies. In this study we present 13 new cases of Merkel cell carcinoma (the largest published series in the UK) and summarize 214 cases from the literature in which the survival data are given. In our series, 5 of 13 patients died from spread of the Merkel cell carcinoma. From this and other studies, it appears that early diagnosis and wide local excision may be the only way to prolong survival. No other adjuvant therapy has proved effective.     

Percutaneous biopsy of the thoracic and lumbar spine: transpedicular approach under fluoroscopic guidance

BACKGROUND: Merkel cell carcinoma (MCC) is a rare, aggressive, cutaneous neoplasm. The primary form of initial treatment is wide surgical excision. The use of Mohs micrographic surgery as the primary form of treatment in MCC has been controversial. The course of MCC is often aggressive, with early metastasis, widespread disease, and death. Despite the poor prognosis, spontaneous regression has occasionally been reported. OBJECTIVE: We describe the clinical course of two patients with Merkel cell carcinoma who underwent treatment with Mohs micrographic surgery for the primary MCC. Metastases were excised in the first case and spontaneously regressed in the second. Both patients are without clinical disease at the time of this report. METHODS: Histopathology, clinical records, and the current literature are reviewed. RESULTS: One patients was without recurrence of MCC for 13 years of follow-up. The other patient experienced clinical spontaneous remission after nodal spread of the disease, with no recurrence for 18 months after clinical remission and 24 months after surgery. CONCLUSION: The treatment of Merkel cell carcinoma with Mohs micrographic surgery (MMS) has been successful for the control of primary skin disease, and is at least comparable to wide excision. Spontaneous regression may occur in the course of this usually relentless and aggressive disease. The explanation for spontaneous regression of MCC is unknown.     

Pagetoid Merkel cell carcinoma: epidermal origin of the tumor

We report a case of intraepidermal Merkel cell carcinoma which occurred on the face of a 76-year-old white male. This slow-growing tumor was mostly confined in the epidermis and pilosebaceous apparatus where tumor cells spread in a pagetoid fashion forming tumor cell nests. Histologically it resembled a superficial spreading melanoma. A heavy lymphocytic infiltration was seen beneath the epidermal lesion as is often seen in pagetoid melanomas. Histochemical and ultrastructural features such as the presence of cytokeratin 20, synaptophysin, neuron specific enolase, desmosomes, and dense cored granules confirmed the diagnosis of Merkel cell carcinoma. Occasional mitotic cells and many apoptotic cells were found in the tumor. Dylon positive, amyloid depositions were seen in the lower epidermis and papillary dermis; they were probably derived from apoptotic tumor cells. It was thought that apoptosis limited the speed of growth of this tumor. We believe that this is probably the most convincing case of intraepidermal Merkel cell carcinoma originating from epidermal Merkel cells or its precursors (stem cells).     

Merkel cell carcinoma in the nasal cavity: a case report

Merkel cell carcinoma is an aggressive dermal neoplasm of neuroendocrine origin with a predilection for the head-and-neck region in elderly patients. We present a case of Merkel cell carcinoma of the nasal fossa. Local wide excision was performed.     

CT characteristics of metastatic disease of the pancreas

Contrast material-enhanced computed tomographic (CT) scans obtained over a 10-year period in 66 patients with metastases to the pancreas were retrospectively reviewed. The primary tumors most commonly responsible for these metastases were renal cell carcinoma (30.3%) and bronchogenic carcinoma (22.7%). Metastases showed no predilection for any particular part of the pancreas. The majority (75.8%) of metastases appeared as tumors with discrete margins, and most of these tumors were round or ovoid with smooth borders. Over three-fourths of the lesions demonstrated enhancement (usually heterogeneous). Vascular involvement was uncommon. In those patients in whom pancreatic metastases were discovered some time after the primary tumor was identified, the interval ranged from 2 to 295 months, with the longest mean interval (120.2 months) being associated with metastatic tumors from renal cell carcinoma. The appearance of these tumors at CT--predominantly hyperattenuating masses, often with nonenhancing internal components--was similar to that of primary renal cell carcinoma. In most pancreatic metastases, however, clinical information in conjunction with CT characteristics such as multiplicity of tumors or hypervascularity permit differentiation of metastases from primary neoplasm. When diagnosis of a pancreatic neoplasm is uncertain, percutaneous biopsy often permits histologic confirmation of the tumor type.     

Merkel cell tumor

Merkel cell cancer is a rare carcinoma arising from the neuroendocrin cells of the skin. The diagnosis is based on the clinical behaviour, histopathologic and ultrastructural findings and immunohistochemical results. An unusual case of Merkel cell carcinoma is presented. Mass from the umbiculus and a right inguinal lymph node was excised in a 63-year-old female. The histologic features of a typical, primitive small cell tumor combined with the immunohistochemical evaluations established the diagnosis. Rare polynuclear giant cells were focally present in our case. Patient was treated with combination of chemotherapy (Cisplatin, Etoposid) and radiotherapy. Control examinations showed complete respond. One year later metastasis developed. Resection of all known metastasis were performed. Two months after the laparotomy she died of metastatic disease. The autopsy did not reveal any other primary tumor. The capricious nature of the clinical course and the differences between this tumor and other carcinomas is emphasized.     

Comparison of the Nebraska collar, a new prototype cervical immobilization collar, with three standard models

This is a retrospective reassessment of the most important cytopathologic features of 23 FNA smears with a cytologic diagnosis of panniculitis (PN). Patients were sent by clinicians. Clinical diagnoses were as follows: 16 suspicious of PN; three cutaneous metastases of an extracutaneous primary neoplasm; four with no clinical diagnosis. Thirteen cases were subsequently submitted to histopathologic study. The following cytoarchitectural patterns were found to be very useful for the cytologic diagnosis of PN: adipocytes intermingled with foamy histiocytes, donut-like granulomas, aggregates of adipocytes intermingled with plump histiocytes, a granular basophilic background forming a lattice-like pattern, and well-formed granulomas with or without multinucleated giant cells. Inflammatory cells could be seen combined with any of these cytoarchitectural patterns. FNA does not pretend to replace excisional biopsy as the diagnostic procedure for these entities but it is a very useful diagnostic tool in certain cases: for confirming the recurrence of PN previously diagnosed by histology, for evaluating the onset of subcutaneous nodules in patients with a non-cutaneous malignant primary neoplasm, for evaluating cutaneous nodules with no clinical suspicion, and for confirming a clinical diagnosis of PN and differentiating it from other entities that mimic PN clinically.     

Merkel cell tumor of the head and neck. Five new cases with literature review

Merkel cell carcinoma (MCC) of the skin is an uncommon, but highly aggressive neoplasm with a marked propensity for local and distant metastasis. Despite the fact that more than half of the 600 cases of MCC reported in the literature involved primary sites in the head and neck, MCC has rarely been discussed in otolaryngology publications. We present five new cases of MCC of the head and neck and summarize 89 additional cases from the literature in which detailed treatment and survival data were given. Our findings again emphasize the difficulty in making the initial histopathologic diagnosis of MCC and demonstrate the necessity of early diagnosis and multimodality treatment.     

Correction of hypospadias

A case of Merkel cell carcinoma of the skin is described. It is a rare primary skin tumor originating within the dermis with an aggressive biologic behavior. Clinical presentation of these tumors are not sufficiently distinctive to allow the preoperative diagnosis. Wide surgical excision with postoperative irradiation to the local site and regional lymphatics is recommended as the therapy of choice. Indications for chemotherapy are discussed and the guidelines for an appropriated follow-up are reported.     

Merkel cell tumor. Report of case and treatment with octreotide

Merkel cell carcinoma is a rare neuroendocrine tumor of the skin. Prognosis is very poor particularly when systemic disease is present. Surgery, chemo and/or radiotherapy treatment are not able to guarantee long survival and quality of life is also very poor. We know that neuroendocrine tumor can be in possession of receptors for somatostatin; during the past years, these receptors have been demonstrated in vivo by octreoscan. We report a case of a patient suffering from metastatic Merkel cell carcinoma; because he was elderly, neither chemotherapy nor radiotherapy were possible as a consequence of the explosion of the disease after surgery. The presence of receptors for somatostatin analogues (octreoscan) allowed treatment with octreotide causing the immediate disappearance of metastasis. After ten months of treatment the patient presents a complete remission of disease. Octreotide, the most important somatostatin analogue, represents a primary role in the neuroendocrine tumor management; the drug also lacks of toxicity.     

Positron emission tomography (PET) methodology for small animals and its application in radiopharmaceutical preclinical investigation

Among the more uncommon tumors that may sometimes be encountered in the laryngeal region is the recently described giant cell tumor of the larynx. This lesion is a true neoplasm, presumably of the fibrohistiocytic series. Histologically, it closely resembles the more familiar true giant cell tumor of long bone. The laryngeal giant cell tumors appear, to date, to be nonmetastasizing lesions; it is possible that they may recur locally if incompletely excised (although this remains to be demonstrated). In view of the rarity of these tumors, a tentative diagnosis of such a neoplasm should always prompt consideration of other (more frequently encountered) differential diagnostic possibilities, including cytologically malignant giant cell-rich tumors such as malignant fibrous histiocytoma and sarcomatoid carcinoma.     

Urothelial carcinoma of the navicular fossa

We report a case of transitional cell carcinoma arising in the fossa navicularis. The patient was a 74-year-old man. He had no history of sexually transmitted disease or urethral stricture. Clinically, the tumor was suspected to be a condyloma acuminatum; however, the pathological diagnosis yielded an unexpected result: transitional cell carcinoma. Radiological examinations and cystoscopy showed no other tumor besides the primary cancer in the fossa navicularis. Partial resection of the urethra was performed and the patient has been without evidence of disease for 3 years.     

Merkel cell carcinoma: primary cutaneous neuroendocrine tumor

Merkel cell carcinoma is a neuroendocrine primitive tumor of the skin. We report a case who develops node and visceral metastasis, and dead. The clinical presentation, diagnosis, histology, immunocytochemistry, treatment and prognosis of this tumor will be discussed.     

Reticulum cell neoplasms of lymph nodes: a clinicopathologic study of 11 cases with recognition of a new subtype derived from fibroblastic reticular cells

Lymph nodes contain nonlymphoid accessory cells including follicular dendritic cells (FDCs), interdigitating dendritic cells (IDCs) and fibroblastic reticular cells (FBRCs). Neoplasms derived from FDCs are uncommon, and those of IDC origin are even more rare. We report the clinicopathologic features of 11 reticulum cell neoplasms, including 2 of FBRC origin. There were seven male patients and four female patients ranging in age from 13 to 73 years. All cases involved lymph nodes (cervical or supraclavicular-6 cases), (abdominal--2 cases), epitrochlear (1 case); two had more than one site of involvement (cervical lymph node and mediastinum--1 case, cervical and abdominal lymph nodes--1 case). One case of FDC tumor had concomitant Castleman's disease, plasma cell variant. Each neoplasm showed similar histology with oval-to-spindle-shaped cells in a storiform or fascicular pattern. Based on immunophenotypic findings, the neoplasms were classified as FDC (five cases), IDC (two cases), FBRC (three cases), and reticulum cell neoplasm, not otherwise specified (one case). The FDC tumors showed immunoreactivity for CD21 or CD35, vimentin, and CD68. The IDC tumors showed strong positivity for S-100 protein and variable positivity for CD68 and CD1a. The cases derived from FBRCs were positive for vimentin, desmin, and smooth-muscle actin. The neoplasm classified as reticulum cell neoplasm, not otherwise specified had similar morphologic features but showed only equivocal positivity for CD68 and vimentin. Follow-up was available for 9 of 11 (82%) cases with a mean of 3.5 years. Four of five patients with FDC tumors were alive with disease when last seen; the fifth is alive and well with no evidence of disease at 4-year follow-up. One patient with IDC tumor had a recurrence in a different nodal site. Two patients with FBRC tumor were disease free at follow-up of 2 years and 8 years, respectively. The patient with reticulum cell neoplasm, not otherwise specified, was alive and disease free 8 years after diagnosis.     

Hernia development in CAPD patients and the effect of 2.5 l dialysate volume in selected patients

Merkel cell carcinoma (MCC) of the skin is a rare, primary malignant skin neoplasm which can present as a cutaneous nodule. These neoplasms are seen primarily in the elderly and located in the head and neck area or extremities. Twenty-nine aspirates from primary and metastatic lesions obtained by percutaneous fine-needle aspiration in 19 patients have been studied. The cytomorphologic features, clinical information, and immunocytochemical (ICC) findings are detailed. Aspirate smears demonstrated small-to-intermediate-sized cells with a loosely cohesive pattern. Nuclei were round with finely granular chromatin and multiple, small nucleoli. Cells possessed a thin rim of cytoplasm, and infrequent pseudorosette formations were noted in cell groups. ICC results were universally positive for cytokeratin, which showed a paranuclear "dot-like" pattern. Neuron-specific enolase, epithelial membrane antigen, and S-100 protein were positive in varying degrees. Leukocyte common antigen was universally negative. The diagnosis of MCC of the skin by FNA can be made by applying cytologic features in addition to ancillary studies and clinical information.     

Immunohistochemical staining of ovarian granulosa cell tumors with monoclonal antibody against inhibin

Inhibin is a peptide hormone produced by ovarian granulosa cells and by granulosa cell tumors. Serum inhibin measurements have been used as a biochemical marker of the presence or progression of ovarian granulosa cell tumors and their metastases. In the current study, an antibody against the alpha-subunit of human inhibin was used to stain 16 cases of ovarian adult granulosa cell tumors, 15 cases of other ovarian sex cord-stromal tumors, and 51 cases of a range of ovarian and extraovarian neoplasms, many of which may mimic granulosa cell tumor. There was diffuse strong cytoplasmic staining of all cases of adult granulosa cell tumor. Diffuse positive staining also was observed in all Leydig cell tumors, and there was focal staining in a proportion of fibrothecomas. There was focal weak staining of one case of ovarian clear cell carcinoma but no staining of other ovarian and extraovarian neoplasms. Immunohistochemical staining with antibodies against inhibin is of value in the diagnosis of granulosa cell tumor and in the distinction of this neoplasm from others that may mimic it. The antibody also may be useful for the confirmation of late metastasis of granulosa cell tumor, especially when the previous history is not known.     

Transjugular intrahepatic portosystemic shunt in an infant

BACKGROUND: Merkel cell (neuroendocrine) carcinoma (MCC) is a very aggressive primary cutaneous neoplasm most often occurring on the head and neck of the elderly. Spontaneous regression of MCC was first described in this journal in 1986. Since then, other such cases have been reported. This case represents the sixth case of spontaneous regression of MCC. OBJECTIVE: To describe to clinical course in a patient with MCC who underwent spontaneous regression of metastatic disease. METHODS: Clinical records including detailed history and frequent follow-up examination made this observation possible. RESULTS: Complete clinically evident regression of metastatic MCC was observed in this case. However, the patient received no treatment known to be effective for MCC. CONCLUSIONS: Spontaneous regression of MCC has been documented. The reason for regression is unknown. Further study of these rare cases may in the future provide more answers than questions.     

Histology, immunohistochemistry, and electron microscopy of small round cell tumors of bone

Small round cell tumors (SRCTs) of the bone make up a family of primary bone sarcomas with morphologically, biologically, and clinically specific features. Among them, Ewing's sarcoma (ES) is the most common entity, but several varieties such as atypical ES, large cell ES, and ES with neuroectodermal differentiation (peripheral primitive neuroectodermal tumor of the bone or neuroepithelioma of the bone) have been identified recently. Histology and electron microscopy together with the variable expression of several epitopes (as shown by immunohistochemistry, mainly HBA/71 [Mic2 antigen]) provide the basis for characterizing the group within the context of neuroectodermal-derived neoplasms. A number of other ES-like tumors with small round cells, mimicking those previously described, have been characterized; Askin's tumor of the thoracopulmonary region will be considered as an ES similar to those already described, but within a particularly anatomic location. On the other hand, the presence of an endothelial appearance within a poorly differentiated neoplasm may be present in some ES-like SRCTs (atypical ES with endothelial features). The differential diagnosis with other sarcomas defined by small round to spindle cell contours might prove difficult. Particular attention must be paid to small cell osteosarcoma and mesenchymal chondrosarcoma. Likewise, "primitive sarcoma of bone" is considered in this study because it is a very rare neoplasm differing from the formerly discussed types; its pluripotentiality provides this tumor a blastemic character and a multiphenotypic expression. Malignant non-Hodgkin's lymphoma is an unusual presentation when primary to the bone, previous to any other anatomic location. Several subtypes have been considered within a histology that encompasses that seen in lymph nodes.     

Early invasive colorectal carcinomas metastatic to the lymph node with attention to their nonpolypoid development

Clinicopathologic study of six cases of early invasive colorectal carcinoma metastatic to lymph node was performed in order to elucidate possible characteristics relating to the risk of metastasis, with particular attention to the growth pattern of the primary tumor. All of the cases had at least one of the well-known risk factors for lymph node metastasis, including moderately or poorly differentiated histologic characteristics, considerable degree of submucosal invasion, and lymphatic invasion. An interesting finding of the present study was the identification of a nonpolypoid growth pattern with no concomitant adenomatous tissue, which seemed to be different from that of "malignant polyps" of previously reported cases showing adenoma-carcinoma sequence. This unique growth feature was found in all of the cases. Therefore, in addition to the accepted risk factors, nonpolypoid growth pattern and absence of adenomatous component may be risk factors predictive of nodal metastasis in patients with early invasive colorectal carcinoma.     

Adenoid cystic carcinoma of the submandibular gland with symptomatic ovarian metastases

We report the clinical and pathologic features of an adenoid cystic carcinoma of the submandibular gland that metastasized to the ovaries 10 years after initial presentation. A 30-year-old woman underwent excision of a right submandibular adenoid cystic carcinoma followed by regional external beam radiation therapy. Three years later, she underwent extended hepatic resection and localized radiotherapy to the hepatic region for metastatic disease. The patient was without evidence of disease for 7 years when she developed pelvic pain and a pelvic mass was found. A solid and cystic 10-cm left ovarian mass and a single metastatic tumor nodule involving the right ovary were excised via the laparoscope. Histologically, the tumor was identical to the patient's initial salivary gland neoplasm. The neoplastic cells were CAM 5.2 positive, S100 positive, muscle-specific actin positive, and smooth muscle actin positive. Ultrastructurally, characteristic pseudocysts (pseudolumina) with abundant basal lamina and true glandular lumina lined by short microvilli were present. Other than a single anecdotal account of a parotid gland adenoid cystic carcinoma, this case represents the first documented report of an adenoid cystic carcinoma of salivary gland origin that was associated with symptomatic ovarian metastases. This case demonstrates that the ovary is a potential site for metastatic disease many years following the diagnosis and treatment for a primary neoplasm however uncommon or remote the site of origin. Since metastatic adenoid cystic carcinoma can rarely present as an ovarian mass, a clinical history of this neoplasm should be heavily weighed in the differential diagnosis of any unusual ovarian tumor with a predominant cribriform, trabecular, or tubular pattern.