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.     

Gangliogliomas: a clinicopathological study of 25 cases and review of the literature

The histopathological, clinical, and radiological findings in 25 patients (median age 20.5 years; range 1.7-64.2 years) with gangliogliomas were assessed to correlate degree of astrocytic anaplasia and proliferative potential with recurrence or survival. Most patients (64%) presented with seizures (median Karnofsky Performance Score 90%; range 70-100%). Computerized tomography and magnetic resonance imaging showed nonspecific abnormalities. Neoplastic ganglion cells were defined as heterotopic, irregularly grouped, or having more than one nucleus of bizarre shape or size. The astrocytic component was moderately anaplastic in 15 cases and highly anaplastic (HAA) in 10. Eight patients had gross total resection, 11 had subtotal resection, and six underwent biopsy. Ten patients (five gross total resection, three subtotal resection, two biopsy) had no further treatment, 15 underwent external irradiation, and five had adjuvant chemotherapy. Twenty-four patients are alive 15-394 weeks (median 203.5 weeks) postoperatively; one with ganglioglioma-HAA died at 65 weeks. No tumor recurred after gross total resection. Duration of preoperative symptoms < 1 year, greater anaplasia, and age > 30 years at diagnosis may have increased the risk of recurrence after subtotal resection or biopsy by four, three, and two times, respectively (not significant). Bromodeoxyuridine labeling index (BUdR LI) was < 1% in eight non-recurring tumors and 1.3% in another recurring twice (second recurrence LI = 1.6%). Most patients with ganglioglioma have a good prognosis. After gross total resection, only observation is required. After subtotal resection or biopsy, recurrence is possible. BUdR labeling may guide further therapy.     

Lingual osseous choristoma: a study of eight cases and review of the literature

A choristoma is a tumor-like mass of normal tissue in an "abnormal" location. Lingual osseous choristoma, previously known as osteoma of the tongue, is a rare entity: we found documentation of eight cases at our center during the 10-year period between 1987 and 1996. These cases were analyzed along with 50 others reported in the English language literature between 1913 and 1996. Lingual osseous choristoma frequently occurs during the third and fourth decades of life, and predominantly affects women (81%). Most of the osseous choristomas in our review were located in the posterior third of the tongue, primarily at or close to the foramen cecum or circumvallate papillae (87.9%). While 39.7% of the patients were asymptomatic, the remaining patients complained of symptoms including a lump in the throat (25.8%), dysphagia (6.9%), gagging (5.1%), nausea (3.4%) and irritation (3.4%). Treatment of lingual osseous choristoma consists of simple excision. The tumor's origin has been discussed elsewhere.     

Malignant mucosal melanoma of the head and neck: review of the literature and report of 14 patients

BACKGROUND: Fortunately, primary malignant mucosal melanoma of the head and neck is a rare entity. A paucity of data elucidating the predictive factors as well as the unpredictable and aggressive biologic behavior of mucosal melanoma compound the vexing clinical situation. This review summarizes what the literature reveals about the epidemiology, patient survival, patterns of local recurrence, and local and distant metastasis of the disease. Over 1000 patients with this disease have been reported. Survivals at 5 and 10 years is 17% and 5%, respectively. Approximately 19% of patients present with lymph node metastasis and another 16% develop lymph node metastases after treatment, whereas 10% present with distant metastasis. Local metastasis does not affect survival; this is in sharp contrast with skin melanoma. Over 50% of patients experience local treatment failure, and salvage treatment is effective in only 25% of these cases. Local failure is the harbinger of distant metastases. Patients with nasal mucosal melanoma have a 31% 5-year survival rate, whereas sinus melanoma patients fare poorly, with a 0% rate of 5-year survival. METHODS: The authors conducted a retrospective review of 14 patients with characteristics similar to those in the literature in terms of outcome. RESULTS: The 5-year survival rate for these patients was 14%. Whole-body positron emission tomography was performed on 3 patients to detect metastatic disease. The patterns of local recurrence, distant metastasis, and survival for these patients were compared with the same data for patients described in the literature. CONCLUSIONS: Surgery appears to have the greatest efficacy in the management of mucosal melanoma, although radiation therapy may play an increasingly important role in the future.     

Myofibrosarcoma of the head and neck in children

We have identified a distinctive malignant soft tissue neoplasm that occurred in the head and neck region of six children. Histologically, these neoplasms presented an array of features ranging from low-grade spindle cell to high-grade fibrohistiocytic histologies and often had myoid characteristics. Ultrastructural and immunohistochemical studies indicated that they contained neoplastic myofibroblasts that were variably positive for vimentin (4 positive/4 tested), alpha-smooth muscle actin (4/5), muscle-specific actin (5/5), desmin (2/5), and v-src protein substrate p80/85 (4/5). Three patients died of rapidly progressive unresectable local disease, one died of metastatic and local disease, and two are alive 13 months and 8 years after wide resection. We conclude that these neoplasms form a distinctive subset of pediatric soft tissue sarcomas that display an aggressive clinical behavior, typically with local recurrence, and exhibit features of myofibroblastic differentiation.     

Granulosa and theca cell tumors in children: a report of 17 cases and literature review

OBJECTIVE: To identify preoperative and perioperative features that may lead to a risk of surgical-site infection (SSI) after coronary artery bypass surgery. DESIGN: 884 patients who underwent coronary artery bypass grafting in 1992 and 1993 were studied. The associations between 23 preoperative and perioperative features and the presence of SSI at the donor site or in the chest area were evaluated by univariate analysis followed by multivariate logistic regression analysis. SETTING: A university hospital. RESULTS: 172 patients (19.5%) either had an SSI recorded before discharge or had received antibiotics prescribed for a suspected SSI during the 1-month surveillance period after discharge. Multivariate logistic regression analysis showed an extreme body mass index (BMI; P=.015), female gender (P=.023), and chronic obstructive pulmonary disease (COPD; P=.030) to be independent risk features for SSI. The donor site was infected in 136 patients (15.4%), an event for which female gender (P=.003) was the only independent risk feature. Forty-seven patients (5.3%) had an SSI of the chest area, with diabetes (P=.003) and extreme BMI (P=.010) as independent risk features. CONCLUSION: Extreme BMI, female gender, and COPD are highly significant independent predictors of the development of SSI. Female gender is a risk feature specifically for SSI at the donor site, whereas diabetes and extreme BMI predict it in the chest area.     

Combined radiochemotherapy for organ preservation in head and neck cancer: review of literature and personal experience

Combined radiochemotherapy is the most common method aimed at improving the rate of clinical response in advanced head and neck cancer. Complete clinical remission may correspond to a significant percentage in organ and/or function preservation. In 1992 a protocol of concomitant radiochemotherapy with continuous infusion of carboplatin for 14 consecutive days at the daily dose of 30 mg/m2 and concomitant radiotherapy with conventional fractionation (1.8 Gy to a total 65-70Gy) was started. Over a 3-year period, 56 patients with advanced head and neck cancer, were treated. In view of organ preservation, 26 patients of this series, though with considerable extent of the disease at diagnosis, were considered candidates for radical surgery: oral cavity 9 patients; oropharynx 9 patients; larynx/hypopharynx 8 patients. A single patient was stage I (hypopharynx); most patients were stage III (7) and IV (17 = 65%); T4 20%, N3 23%. 17/20 patients (70%) showed complete clinical response, 6 partial clinical response with a single non responder (overall response 95%). A patient underwent total glossectomy followed by local recurrence and another patient underwent pharyngolaryngectomy also followed by recurrence. After a mean follow-up from 22 to 60 months, 9 patients were still free of disease (37.5%). Median duration of complete response was 25.6 months. Overall median survival was 26.7 months: 38 months in responders. 2-year survival of patients with complete response was 59%. As for organ preservation, at present 6 over 18 patients (33%) with tumor of the oral cavity or oropharynx and 3 patients with tumors of larynx/hypopharynx have preserved organ and function. As for complete responders, 54.5% of those with tumors of oral cavity or oropharynx and 50% of those with tumors of larynx/hypopharynx, have preserved anatomy and function after at least 2-year follow-up. To-date, in follow-up controls relevant late toxicity has not been observed, showing that to the positive anatomical result corresponds the functional preservation of single structures.     

Imaging of pediatric head and neck rhabdomyosarcomas with emphasis on magnetic resonance imaging and a review of the literature

In the mouse, the initiation of the attachment reaction between the blastocyst trophectoderm and luminal epithelium of the receptive uterus occurs in the evening (2200-2300 h) of day 4 of pregnancy (day 1 = vaginal plug) and is followed by proliferation and differentiation of stromal cells into decidual cells at the sites of blastocyst attachment. This investigation demonstrates that an inappropriate expression of the human transforming growth factor alpha (hTGF-alpha) transgene in the uterus under the direction of a mouse metallothionein-I promoter downregulates uterine expression of TGF-beta receptor subtypes and delays the initiation of implantation (attachment reaction) resulting in delayed parturition. This delay in the attachment reaction is accompanied by deferred uterine expression of amphiregulin. The results suggest that a coordinated 'cross-talk' between the signaling pathways executed by epidermal growth factor-like growth factors and TGF-beta 5 is important for the normal implantation process.     

Dermoid cyst of the lateral neck: a case report and literature review

Dermoid cysts of the lateral neck are rare, with the majority of head and neck dermoids occurring in the midline. The demonstration of a fat-fluid level on MRI or CT is diagnostic for a cervical dermoid cyst. The treatment of choice remains surgical excision.     

Salivary duct carcinoma: a clinicopathologic study of three cases with a review of the literature

Three cases of salivary duct carcinoma are presented. They occurred in a 60 year old man, a 66 year old man and a 57 year old woman. All of the lesions were located in the parotid gland. The tumor size ranged from 3 to 5 cm across the largest diameter. Facial paralysis was observed in two cases. Histologically, intraductal and invasive adenocarcinoma showing papillary, cribriform, and solid patterns with comedolike necrosis was observed. Immunohistochemically, the tumor cells were positive for keratin and epithelial membrane antigen. No myoepithelial cells were demonstrated within the tumor by staining for S-100 protein, alpha-smooth muscle actin or muscle specific actin. Ultrastructurally, intracytoplasmic lumina with microvilli, a moderate number of mitochrondria, lysosomes, and tight junctions were found. Regional lymph node metastasis was observed in one case, and distant metastasis developed in two cases. All of the patients were treated with adjuvant postoperative irradiation. One patient died of disease at 11 months after the initial diagnosis, another was alive with disease at 8 months, and the third patient was alive without disease at 2 years and 3 months. Salivary duct carcinoma should be differentiated from low-grade salivary gland carcinomas using morphologic and clinical criteria because of its poor prognosis even with aggressive therapy.     

Central neurocytoma: clinico-pathological study of 5 cases and review of the literature

Five cases of central neurocytoma, confirmed by immunohistochemical and electron microscopy studies, are reported and 127 cases from the literature are reviewed. Central neurocytomas are more frequent than previously thought, and will be diagnosed with increasing frequency in the future, if intraventricular tumors with histological aspect of oligodendroglioma or ependymoma will be routinely studied by immunohistochemistry and electron microscopy. The occurrence of an intraventricular tumor with a typical MR aspect in a young patient should suggest preoperatively the diagnosis of neurocytoma. The positivity for synaptophysin and neuron specific enolase, the negativity for neurofilament protein and glial fibrillary acid protein, and the finding of elements of neuronal differentiation on electron microscopy, are the main pathological features of these tumors. Complete removal of the tumor mass without radiotherapy is the treatment of choice. The prognosis is usually favorable without recurrence.     

Megalourethra: a report of four cases and review of the literature

Megalourethra is a rare congenital anomaly characterized by severe dilatation of the penile urethra. Four cases of congenital megalourethra were seen at Sir Padampat Mother and Child Health Institute, Jaipur, during the last 10 years. Three cases of scaphoid megalourethra had no other associated congenital anomalies and were treat-ed successfully without any complications, while one patient with a fusiform megalourethra had severe associated congenital anomalies and died. These cases are reported with a review of the literature.     

Nonstandard fractionation schedules in radiation therapy of head and neck cancer: a review

The authors present an updated review of the clinical trials on hyperfractionated and accelerated fractionation schedules in radiotherapy of head and neck cancer. The available results in terms of survival and local control, and acute and late toxicity data are summarized in order to show the current status of this research field. The new breed of fractionation schedules that are on study, designed on the ground of new rationales, are presented as well. Finally, an introductory overview of combination therapy including non standard fractionation radiotherapy associated with chemotherapy is reported.     

Leri's melorheostosis: three pediatric cases and a review of the literature

Three pediatric cases of melorheostosis are described. Growth disorders of the limbs are often the first signs in children. The radiological appearance consists of long tracks of increased cortical radiodensity in the long bones and of small dense islets in the epiphyses.     

Angiosarcoma of the head and neck managed by a combination of multiple biopsies to determine tumor margin and radiation therapy. Report of three cases and review of the literature

BACKGROUND: Cutaneous angiosarcoma (AS) is a rare, often multicentric vascular tumor of the head and neck region with a rather poor prognosis. The original clinical size of the tumor rarely correlates to the degree of microscopic tissue invasion. Treatment by surgical excision sometimes requires very wide excision. Treatment by radiation or electron beam appears less mutilating but its efficacy is not well documented. OBJECTIVE: To present our experience with a combined surgical delineation of tumor margins followed by radiation treatment. METHODS: We treated three patients with extensive AS of the scalp and face. Prior to radiation, in two cases the tumor margins were determined by grid-pattern punch biopsies. In the third patient, the tumor margins were determined by Mohs mapping system. All three patients then received radiation either by rotational arc electron beam (n = 2) and standard radiation. RESULTS: One patient developed two local recurrences in nonirradiated areas plus a metastatic cervical node, all of which responded to additional electron beam. The patient has no evidence of disease (NED) after 30 months of observation. The other two patients were treated by electron beam and radiation have NED at 5 and 1 years follow-up, respectively. CONCLUSIONS: Local control of AS of the scalp may be achieved by assessment of the tumor margin by peripheral biopsies or Mohs technique followed by electron beam and radiation.     

Ureterovaginal fistula: a report of 12 cases and review of the literature

Ureterovaginal fistula is an uncommon complication of pelvic operations, seen most often after Wertheim's hysterectomy. We report 12 cases of ureterovaginal fistulas seen during a 20-year period, all of which followed operations for benign gynecologic conditions. Most patients had no urinary symptoms until the sudden onset of incontinence 1 to 4 weeks postoperatively. Diagnosis was established readily by a combination of excretory urography, cystography, cystoscopy, retrograde pyeloureterography and dye studies. In our series only 1 patient was treated by primary nephrectomy, while 11 underwent ureteroneocystostomy: 2 with a Boari flap and 9 by a direct method. Reconstruction failed in 2 patients, 1 of whom required a secondary nephrectomy.     

Parameatal cyst: presentation of two cases and a review of the literature

OBJECTIVE: To describe two additional cases of parameatal urethral cyst. The literature is reviewed and the etiology and treatment of this disease are discussed. METHODS: Two patients aged 20 and 24 years with parameatal urethral cyst are described. The patients were seen at the Gea González Hospital (México) during the period 1987-1988. The first case was asymptomatic and the cyst had been present since birth and in the other case, the cyst appeared at age 24 and caused irregular stream. Laboratory tests, surgical excision and pathological analysis of the cyst were performed. RESULTS: The laboratory tests were normal, no problems were encountered during surgical excision, the symptoms disappeared and there were no postoperative complications or recurrence. Pathological analysis demonstrated columnar epithelium in both cases. CONCLUSIONS: Parameatal urethral cyst is a rare benign condition that is asymptomatic in most of the cases. It may be present since birth or appear later and is prevalent in young males. Its etiology remains unclear and treatment is by complete surgical excision to avoid complications and recurrence.     

Atypical ameloblastoma: report of 3 cases and a review of the literature

The aim of our study was to test the hypothesis that the better absorption of sorbitol when ingested with glucose could be related to a delayed gastric emptying. We tested the effect of the ingestion of glucose and lipids on the gastric emptying and intestinal absorption of sorbitol in six healthy volunteers, using gastric scintigraphy and hydrogen breath test. After an overnight fast, subjects ingested in random order, on 48-h test periods separated by at least one week, the following solutions: (a) 20 g sorbitol alone; (b) 20 g sorbitol and 20 g glucose; (c) 20 g sorbitol and 9 g lipids. Isotopic acquisitions were taken for 3 h following the ingestion of sorbitol labelled with 111Indium. Hydrogen concentration was measured in end-expiratory samples during 5 h, and the areas under the breath hydrogen curve, reflecting the amounts of sorbitol unabsorbed in the small bowel, were compared between periods. Mean area under the curve was 397 +/- 159 when sorbitol was ingested alone, and this was significantly lower when ingested with glucose or lipids (313 +/- 181 and 337 +/- 135, respectively; P < 0.05). The three curves of sorbitol gastric emptying differed significantly from each other, the gastric emptying being the slowest for sorbitol plus lipids, and the fastest for sorbitol taken alone. We found a positive correlation between the half-emptying time and the hydrogen areas under the curve (r = 0.46, P = 0.05). In conclusion, our study demonstrates that adding glucose or lipids to a solution of sorbitol slows the gastric emptying of sorbitol, resulting in a better intestinal absorption of sorbitol.     

Pitfalls in the diagnosis and management of papillary muscle rupture: a study of four cases and review of the literature

Four cases of papillary muscle rupture occurring in the setting of acute myocardial infarction are presented, which illustrate the following points: the diagnosis may not be apparent at presentation, a mitral regurgitant murmur may be absent, transesophageal echocardiography may establish the diagnosis when transthoracic echocardiography does not, and appropriate surgical correction can lead to excellent functional recovery.     

Paragangliomas of the head and neck region: a clinical study of 69 patients

Over a 38-year-period 73 paragangliomas from the head and neck region were seen at Memorial Hospital. These occurred in 69 patients. There were 44 carotid body, 13 vagal body, eight jugulo-tympanic, and three nasal paragangliomas. In addition, one each arose in the orbit, larynx, and area of the aortic arch. Sixty-two patients were surgically treated while three received irradiation only and four were observed but not treated. Follow-up was obtained for 94% of the patients. There were six postoperative deaths, five of which occurred before 1945. Of those treated surgically, 37 had no evidence of recurrent tumor while the paragangliomas recurred locally in 11. All patients in the radiation and untreated groups had persistent tumor. Malignant behavior with death due to tumor was observed in five cases (four carotid body and one vagal body paraganglioma). Using modern techniques carotid body and vagal body paragangliomas can usually be managed by surgery alone. Other paragangliomas of the head and neck due to their anatomic location are sometimes best treated by a combination of surgery and radiation or only by radiation therapy.