Smooth muscle neoplasms of the urinary bladder: a clinicopathologic comparison of leiomyoma and leiomyosarcoma

We report the clinicopathologic, immunohistochemical, and DNA ploidy findings of 18 leiomyosarcomas of the urinary bladder. In addition, we compare these malignant smooth muscle tumors with 10 cases of urinary bladder leiomyoma. The 14 male and four female patients with leiomyosarcoma ranged in age from 25 to 88 years (mean 64 years). The tumors ranged from 3.0 to 15.0 cm (mean 7.1 cm) in greatest dimension and were moderately to highly cellular, consisting of interlacing fascicles of spindled cells with mild to marked nuclear atypia. Coagulative tumor necrosis was identified in 14 cases (78%), and mitotic activity ranged from 1 to 42 mitotic figures (MF) per 10 high power fields (HPF) (mean 12 MF/10 HPF). Tumors were classified as either high-grade (12 cases) or low-grade (six cases) based on nuclear atypia, mitotic activity, and tumor necrosis. Actin positivity was present in 15 tumors (83%), and desmin immunoreactivity was present in seven tumors (39%). All cases were negative for epithelial markers and S-100. Proliferative activity, as assessed by MIB-1 staining, ranged from 0.1% to 51.4% (median 9.1%). Seven (39%) of the leiomyosarcomas were DNA aneuploid, eight (44%) were tetraploid, and three (17%) were diploid. Five patients underwent radical cystoprostatectomy, one radical cystectomy, seven had partial cystectomy, two underwent pelvic exenteration, and three patients had transurethral resection only. Follow-up information was available on all 18 cases and ranged from 2 to 68 months (mean 22 months). Of the 12 patients with high-grade tumors, six (50%) died of disease from 2 to 20 months (mean 7 months) after diagnosis and three patients (25%) are alive with metastatic tumor. Two of the six patients with low-grade leiomyosarcoma died of tumor, 61 and 68 months after diagnosis. There were five male and five female patients with leiomyoma ranging in age from 22 to 78 years (mean 61 years). The tumors ranged from 0.5 to 4.5 cm (mean 1.6 cm) in greatest dimension, were well circumscribed, and had low cellularity. Mitotic activity, necrosis, and cellular atypia were absent, and the tumors were strongly positive for both actin and desmin. MIB-1 staining ranged from 0% to 3.8% (median 0.8%). Seven (87.5%) of the leiomyomas were DNA diploid or near-diploid and one (12.5%) was DNA aneuploid. Six patients were treated with transurethral resection and four with partial cystectomy. All 10 patients were alive at the last follow-up (mean follow-up 75 months), and no tumor recurred or metastasized. Our study shows that low-grade leiomyosarcomas are capable of malignant behavior, and high-grade leiomyosarcomas appear to behave more aggressively than low-grade tumors. In addition, the diagnosis of urinary bladder leiomyoma should be reserved for noninfiltrative smooth muscle tumors lacking mitotic activity, cytologic atypia, and necrosis.     

Nasal lymphoma. A clinicopathologic study with immunophenotypic and genotypic analysis

We studied 13 cases of malignant lymphoma involving the nasal cavity, in six men and seven women, from 27 to 92 years of age (mean, 56 years; median, 55 years). All lymphomas had a diffuse pattern, with 10 of large-cell type (six immunoblastic polymorphous, one immunoblastic, three large cleaved cell), one of mixed small- and large-cell type and one of small cleaved-cell type. One case could not be subclassified. Angioinvasion and prominent necrosis were seen in 10 cases. Pseudoepitheliomatous hyperplasia of the overlying epithelium was present in five cases. Immunohistochemical studies on frozen or paraffin sections in nine cases revealed that the atypical cells were T cells in four cases (CD8+ in two cases) and B cells with monotypic immunoglobulin in two cases. In three cases, the findings were suggestive but not diagnostic of T lineage. Genotypic analysis in one of two cases of T-cell lymphoma revealed clonal rearrangement of the genes for beta and gamma chains of the T-cell receptor. Patients were treated initially with local radiation therapy (10 cases) or with radiation and chemotherapy (three cases). Eight patients (62%) had no relapse and were free of disease between 9 months and 23 years (mean, 6 years 5 months; median 2 years 1 month) after diagnosis. Five patients developed recurrent disease, three of whom were successfully salvaged. One patient was alive with tumor at the time of last follow-up and one died with tumor. Among cases of malignant lymphoma presenting with involvement of the nasal cavity, we find a high proportion of angioinvasive, diffuse large-cell lymphomas, with a predominance of T-cell type, and a relatively good prognosis when treated with radiation therapy.     

Use of the EEA stapling instrument for control of bleeding esophageal varices

Transection of the esophagus with the EEA stapling device (United States Surgical Corp.) has been used to treat 20 patients with bleeding esophageal varices. Their ages ranged from 38 to 73 years (mean 56.7 years). Sixteen patients (80%) had La?nnec's cirrhosis. A previous occluded portosystemic shunt was present in five patients. Based on Child's classification, there were one class A patient, 18 class C patient, and one patient who was unclassified. Five patients underwent elective operation; four survived (80%). The cases of the remaining 15 patients were treated as emergencies or semiemergencies after intensive preoperative intervention including intravenous administration of vasopressin (Pitressin) (100%), balloon tamponade in 11 (73%), and sclerotherapy in three (20%) failed to control the hemorrhage adequately. None of the patients had rebleeding from varices during the postoperative period. However, 11 of the 15 patients (73%) died. All deaths were related to liver failure, except for two patients who died of irreversible acidosis secondary to shock. Technical difficulty was encountered six times with use of the stapling device, resulting in three perforations of the esophagus, which were recognized and repaired at the time. No deaths were attributed to this complication. Of the eight patients who survived the early postoperative period, three have since died--two of variceal hemorrhage and the other of hepatic failure. Two others have had recurrent variceal hemorrhage controlled by sclerotherapy. The duration of follow-up ranges from 10 to 60 months (mean 31 months). Transection of the esophagus with the EEA stapling device for acute variceal hemorrhage is associated with an excessively high mortality rate despite the fact that it controls variceal hemorrhage. It appears to be of value when used on an elective basis for selected patients who cannot benefit from other modes of treatment.     

Differential diagnosis of occult primary malignant melanoma]

Controversy exists in literature about therapy and prognosis of malignant melanoma of unknown primary site. We investigated frequency, differential diagnosis and follow-up of patients with occult primary malignant melanoma treated at the University of Leipzig in 1996. Among 135 patients with malignant melanoma (MM) seven were found without known primary. In two of seven cases the medical history pointed to regression of primary lesion of skin. In another two cases the diagnosis "melanoma" was changed to "lung cancer" after autopsy and in one case there was a relationship to a naevus blue resected nine months before. Recurrences or metastases occurred within six months after therapy. Two patients are still alive free of disease after a follow up of 33 and 24 months. Five patients died from tumor progression between 2 to 14 months postoperatively. Pitfalls in differential diagnosis and ways to find out the primary are discussed. Patients with unknown primary malignant melanoma should be treated similar to those with known primary. Radical surgery is indicated because it's impossible to determine the prognosis of patients with unknown primary malignant melanoma.     

The neodymium-YAG laser and gastrointestinal malignancy.

Thirty-seven patients with either bleeding or obstructive metastatic gastrointestinal malignant neoplasms were treated with the neodymium-YAG laser between June 1985 and December 1988. The age range for the group was 55 to 99 years, with a mean of 71 years. There were 25 upper gastrointestinal lesions, including 22 obstructive lesions (20 esophageal and two prepyloric gastric) and three bleeding lesions (one metastatic melanoma to the stomach, one duodenal, and one pancreatic carcinoma). Of the esophageal tumors, three were proximal, eight were middle, and nine were distal third. The mean number of laser treatments was 2.6, and the overall survival ranged from 1 to 20 months, with a median of 8 months. The dysphagia grade was improved overall but depended on the site of the tumor. All bleeding lesions were successfully photocoagulated. Twelve colorectal malignant neoplasms were treated palliatively, six for obstruction (three intraperitoneal colon and three rectal) and six for bleeding (three intraperitoneal and three rectal). The mean number of treatments for the group was 1.5, and the overall survival ranged from 2 to 38 months, with a median survival of 15 months. The neodymium-YAG laser was found to be effective as a palliative mode of therapy for the management of malignant gastrointestinal lesions.     

Primary ano-rectal melanoma

Primary malignant anorectal melanoma is an uncommon disease that accounts for 1% of anorectal malignancies. Its virulent malignancy is associated with a poor prognosis and with difficult diagnostic and therapeutic problems. The operative management of these patients is controversial. Clinicopathologic features and surgical treatment of 6 patients with primary anorectal melanoma were studied retrospectively. There was a male preponderance (2:1) with a mean age of 62 years (range: 34-74). The site of origin of the melanoma was rectal in one patient and in the anorectal junction in five patients. Atypical intramucosal melanocyte proliferation was associated with rectal melanoma. The maximum tumor size from 2 to 5.5 cm. Common initial symptoms were rectal bleeding and/or tenesmus. CT was useful for tumor staging. Two patients had distant metastases at initial presentation. Four patients underwent "curative" treatments by abdominoperineal resection and 2 by local excision. The survival for the group as a whole was poor (mean: 12.6 months; range: 7-30 months). Surgery is the primary option. The prognosis, however, is poor, since metastatic disease is commonly established at presentation. Atypical intramucosal melanocyte proliferation may be a marker in association with tumor sited in the rectum.     

Bronchopulmonary carcinosarcoma.

Carcinosarcoma accounted for 0.27% of nearly 3000 lung cancers examined in this department. All the patients were men aged between 44 and 62 years, and a majority of the tumours occurred in the left lung. Three patients died within six months of lung resection and in each case a postmortem examination was performed. In seven the carcinomatous component was a squamous-celled growth, and in one columnar-celled; the histology of the sarcomatous element varied. Carcinosarcomas form a distinct group of malignant lung tumours. In five cases sarcomatous transformation of the stroma had occurred and was considered to be the usual means by which the mixed type of growth arises. The other three were considered to be "collision" tumours. Carcinomatous metastases without sarcomatous change were seen in lymph nodes in three cases, and in three fatal cases sarcomatous tumour had recurred.     

Desmoplastic malignant melanoma and its variants. A study of 45 cases

Forty-five tumors exhibiting the histological features of desmoplastic malignant melanoma or its variant, neurotropic melanoma, were found among approximately 4,500 soft-tissue tumors referred in consultation from Australia and New Zealand. All patients were Caucasians. Tumors fell into three groups: (a) desmoplastic melanoma with an atypical intra-epidermal melanocytic component (classical desmoplastic melanoma) (12 cases); (b) desmoplastic melanoma without an atypical intra-epidermal melanocytic component (de novo desmoplastic melanoma) (21 cases); (c) predominantly nerve-centered superficial malignant tumors with or without an atypical intra-epidermal melanocytic component (12 cases). Three of the nerve-centered tumors were associated with pigmentary abnormalities in the overlying skin. The patients' ages ranged from 42 to 91 years, with a peak in the seventh decade; 31 patients were male and 14 were female. Lesions were located in the head and neck (35 cases), shoulder and arm (four), back and chest (three), abdomen (one), thigh (one), and leg (one). Three tumors arose in irradiated areas, and one occurred on the face of a radiotherapist. Melanin was found in only four tumors, but the S-100 protein stain was positive in 19 tumors and negative in three. Follow-up details of 42 patients were available. In follow-up times from 4 months to 15 years (mean, 4.6 years), 15 patients (36%) had died of the disease or were terminally ill; 24 (57%) were alive with no apparent residual tumor; and three (7%) had died of other causes. Twenty-three patients (55%) developed one or more local recurrences; 17 (40%) developed distant spread, including extension into the cranial cavity along nerves; and 14 (33%) had both local recurrence and distal spread. Nine (82%) of 11 patients with nerve-centered tumors died or were terminally ill. The usual treatment was surgical. Most uncontrolled recurrences were resistant to chemotherapy and radiotherapy; however, two patients responded well, one to chemotherapy and one to radiotherapy. Our observation support Reed and Leonard's metaplastic explanation for the varied differentiation seen in desmoplastic melanomas.     

Cardiac tumors. Clinical spectrum and prognosis of lesions other than classical benign myxoma in 20 patients

Between 1961 and 1983, 20 patients underwent operation at the Texas Heart Institute for primary tumors other than classical benign myxoma. Total follow-up was 70.7 patient years (mean 3.5 years). There were 10 adults and 10 children under 12 years of age. There were two operative deaths and four late deaths. All of the pediatric patients had benign lesions (five fibromas, five rhabdomyomas), and only one patient in this group died (during the operation). All operative survivors are alive between 0.9 and 18 years postoperatively, although in three cases excision was incomplete. Of the 10 adult patients, five had benign lesions; all were completely excised. In the other five adult patients, unresectable malignant tumors were found, and all four operative survivors died of metastatic disease within 8 months. Benign cardiac tumors in childhood have an excellent prognosis when completely excised and appear to have a good short-term prognosis even when excision is incomplete. Although prognosis for benign tumors in adults is good, malignant tumors are associated with very poor survival.     

A clinical study of six surgically treated patients with malignant tumors arising from chronic pleuritis and pyothorax

During the clinical course of chronic pleuritis and pyothorax, we frequently have found abnormal clinical signs such as increased abnormal chest shadows, subjective symptoms, such as chest pain and bloody sputum, and the appearance of atypical cells in the pleural effusion. It is very difficult and important to diagnose differentially worsening of chronic inflammation and occurrence of malignant tumors. We have surgically treated six patients with malignant tumors arising from chronic pleuritis and pyothorax, who had the above abnormal signs. All of them were made, and their average age was fifty three years. Two patients exhibited increased abnormal chest shadows, 5 had subjective symptoms (5 with chest pain, 2 with bloody sputum and 2 with fever), and 1 had atypical cells in the pleural effusion. Two patients had suffered from chronic pleuritis, who both had received artificial pneumothorax therapy for pulmonary tuberculosis, and the other 4 patients had suffered from chronic pyothorax, with mean duration of thirty years. The pathological diagnoses were 2 cases each of malignant pleural mesothelioma, squamous cell carcinoma, and malignant fibrous histiocytoma. The two patients with squamous cell carcinoma had pleurocutaneous and bronchopleural fistula. The operative methods were tumor resections with pleuropneumonectomy performed in 2 patients and pneumonectomy in 1. Absolute non-curative operation was performed in 1 patient, and postoperative radiotherapy in 3 patients. All patients died of tumor recurrence, although five of them underwent reoperation, chemotherapy and radiotherapy. Their duration of survival ranged from 3 to 15 months with a mean of 9 months after appearance of malignant tumor signs.(ABSTRACT TRUNCATED AT 250 WORDS)     

Islet cell carcinoma of the pancreas

To define the course of malignant pancreatic islet cell tumors, 20 patients seen over 14 years with these neoplasms were reviewed. The 12 men and 8 women ranged in age from 22 to 76 years, with a mean of 52. Seven functional tumors included three insulinomas, two glucagonomas, one gastrinoma, and one somatostatinoma. One insulinoma was associated with a multiple endocrine neoplasia type I (MEN-I) syndrome. The 13 patients with nonfunctioning tumors had abdominal pain (3), jaundice (2), and steatorrhea (2). Seven had a palpable abdominal mass. Diagnosis of malignancy was based on local invasion (4), distant metastases (15), or both (1). One patient had percutaneous biopsy of a hepatic metastasis. All others had laparotomy for diagnosis and/or treatment. Each patient had a single tumor except the patient with MEN-I syndromes, who had multiple tumors throughout the pancreas. The head was involved in seven patients, the body in seven, and the tail in five. Operations included six curative and three palliative resections, five biliary diversions (two with concomitant enteric bypass), and five biopsies. Palliative resections were done for hormonal or local symptoms such as gastrointestinal (GI) bleeding and pain. Multiple chemotherapeutic agents were used, but the best results were obtained with DTIC (50% response). Four patients had radiation for liver, brain, or bone metastasis, with some improvement. Of five patients who had curative resections, four are alive 15 to 144 months, with a mean of 75 months. One died six years after diagnosis. Of the remaining 15 patients who had liver metastasis, seven patients are alive 8 to 168 months later, with a mean of 87 months.(ABSTRACT TRUNCATED AT 250 WORDS)     

Sentinel lymph node biopsy in patients with diagnostically controversial spitzoid melanocytic tumors.

Melanomas can be difficult to diagnose histologically if they deviate in their growth pattern or cytology only minimally from a nevus. On occasion, even experts on melanocytic lesions may not reach a consensus on whether a lesion is a benign but unusual nevus or a malignant melanoma mimicking a nevus. This diagnostic dilemma is particularly well known for the distinction of Spitz nevus from melanoma. Diagnostic uncertainty and disagreement among consultant pathologists lead to confusion about the prognosis and clinical management of patients. In this study we present the clinical and pathologic findings of 10 patients with diagnostically controversial melanocytic tumors, who underwent sentinel lymph node biopsy. In all of these cases, the diagnostic controversy among experts was between Spitz nevus and melanoma. Seven patients were female, and three were male, ranging in age from 7 to 46 years (mean 21 years). Histologic examination of the sentinel lymph nodes revealed tumor deposits in the lymph node parenchyma in 5 of 10 patients. Among patients with positive sentinel lymph nodes, two had satellite nodules and one showed additional tumor deposits in three nonsentinel regional lymph nodes. All patients are alive and free of disease with a follow-up of 10-54 months (mean 34 months). Our study illustrates the role of a sentinel lymph node biopsy in the evaluation of patients with diagnostically controversial melanocytic tumors. Although the presence of metastatic tumor deposits in the sentinel lymph node supports the diagnosis of malignant melanoma, further studies are needed to determine the prognostic significance of the sentinel lymph node findings in such patients.     

Vascularized proximal fibular epiphyseal transfer for distal radial reconstruction

BACKGROUND: Treatment of the loss of the distal part of the radius, including the physis and epiphysis, in a skeletally immature patient requires both replacement of the osseous defect and restoration of longitudinal growth. Autologous vascularized epiphyseal transfer is the only possible procedure that can meet both requirements. METHODS: Between 1993 and 2002, six patients with a mean age of 8.4 years (range, six to eleven years) who had a malignant bone tumor in the distal part of the radius underwent microsurgical reconstruction of the distal part of the radius with a vascularized proximal fibular transfer, including the physis and a variable length of the diaphysis. All of the grafts were supplied by the anterior tibial vascular network. The rate of survival and bone union of the graft, the growth rate per year, the ratio between the lengths of the ulna and the reconstructed radius, and the range of motion of the wrist were evaluated for five of the six patients who had been followed for three years or more. RESULTS: The mean duration of follow-up of the six patients was 4.4 years (range, eight months to nine years). All six transfers survived and united with the host bone within two months postoperatively. The five patients who were followed for three years or more had consistent and predictable longitudinal growth. Serial radiographs revealed remodeling of the articular surface. The functional result was rated as excellent for all but one patient, in whom the distal part of the ulna had also been resected because of neoplastic involvement. No major complication occurred at the recipient site, whereas a peroneal nerve palsy occurred at the donor site in three patients. The palsy was transient in two patients, but it persisted in one. No instability of the knee joint was observed. CONCLUSIONS: After radical resection of the distal part of the radius because of a neoplasm in children, vascularized proximal fibular transfer, based on the anterior tibial artery, permits a one-stage skeletal and joint reconstruction, provides excellent function, and minimizes the discrepancy between the distal radial and ulnar lengths.     

Regional perfusion for recurrent malignant melanoma of the extremities

Seventeen patients with a local recurrence or intransit metastases of a malignant melanoma of the arm or leg were treated by normothermic regional perfusion with phenylalanine mustard. Seven of these patients had regional lymph node metastases as well. The follow-up period was either five to eight years or until the time of death. Two years after treatment, eleven of the seventeen patients were still alive. After a five year follow-up, six patients were still alive without demonstrable recurrence or metastases. Only three patients had a recurrence in an extremity; two of these three patients simultaneously showed general metastization.     

The radiotherapy of melanoma.

A retrospective review of a series of cases of malignant melanoma is reported. Two hundred and forty-six records of patients irradiated from 1958 to 1976 inclusive reveal a sex ratio of males to females of 1.59 to 1, with mean ages of 52.4 years and 54.4 years respectively. Three patients are lost to follow-up. Two hundred and twenty are deceased. Of 23 living, nine are diseased and 14 are well. Of 60 patients treated aggressively, 10 survived five years: one of 26 irradiated only; three of 11 irradiated before surgery; and six of 23 irradiated after surgery. Of 168 treated by radiotherapy for palliation of growth restraint, one died of disease after five years, and two are alive and well after 10 years! The primary site was treated in 20 cases, with three patients surviving five years. Secondary sites were treated in 226 cases, with 10 patients surviving five years.     

THE RADIOTHERAPY OF MELANOMA

A retrospective review of a series of cases of malignant melanoma is reported. Two hundred and forty-six records of patients irradiated from 1958 to 1976 inclusive reveal a sex ratio of males to females of 1.59 to 1, with mean ages of 52.4 years and 54.4 years respectively. Three patients are lost to follow-up. Two hundred and twenty are deceased. Of 23 living, nine are diseased and 14 are well. Of 60 patients treated aggressively, 10 survived five years: one of 26 irradiated only; three of 11 irradiated before surgery; and six of 23 irradiated after surgery. Of 168 treated by radiotherapy for palliation of growth restraint, one died of disease after five years, and two are alive and well after 10 years! The primary site was treated in 20 cases, with three patients surviving five years. Secondary sites were treated in 226 cases, with 10 patients surviving five years.     

Anorectal amelanotic melanoma

Objective  Anorectal melanoma is a rare, highly malignant tumour with a poor 5 year survival of 10%. Most anorectal melanomas have gross and/or histologic pigmentation, however about 30% of anorectal melanomas are amelanotic.
Method  We report three cases of amelanotic anorectal melanomas and integrate our data with six case reports of amelanotic malignant melanoma from the literature. Further we compare clinicopathological data and clinical outcome with large series of anorectal melanomas (both, amelanotic and pigmentated).
Results  There were seven females and two males, of median age 62 years (range: 45–75 years). Rectal bleeding was the leading symptom in all cases with a mean duration of 4 months before diagnosis. Eight of nine patients developed distant metastases. Median survival was 14 months (range: 3–60 months). A tumour thickness of < 4 mm was correlated with long-term disease-free survival, whereas tumour thickness of 4 mm or more was correlated with systemic recurrence.
Conclusion  Early diagnosis is key for efficient treatment and improved survival rate for patients with this unusual variant of melanoma. There is no difference in terms of age, time of diagnosis, stage and survival between pigmented and amelanotic anorectal melanoma.     

Pelvic reconstruction with a structural pelvic allograft after resection of a malignant bone tumor

BACKGROUND: Reconstruction of the pelvic arch after resection of a malignant pelvic tumor remains a major surgical challenge because of the high rate of associated complications. The purpose of this investigation was to assess the functional outcome and complication rate following treatment with a bone allograft to reconstruct the pelvis. METHODS: Twenty-four consecutive patients underwent excision of a malignant pelvic bone tumor and reconstruction with a pelvic bone allograft. The living patients were followed for a minimum of twenty-four months. There were nineteen primary malignant bone tumors, sixteen of which were high-grade sarcomas, and there were five isolated metastases. Patients were examined clinically and radiographically and were assessed functionally with the Musculoskeletal Tumor Society score. RESULTS: The mean age of the patients at the time of the index surgery was thirty-four years, and the mean duration of follow-up was forty-one months. Eighteen of the twenty-four resections involved the periacetabular area and were followed by reconstruction either with a hip prosthesis (thirteen) or with an osteochondral allograft alone (five). The six other resections involved the iliac bone. All patients received a massive bone allograft that had been sterilely procured without secondary irradiation. At the time of our last evaluation, eight patients were alive and free of disease. Seven patients had a local recurrence. Neurological deficits were present in six patients, and three had a deep infection. Nonunion of three of the sixteen allografts that could be evaluated was observed. Neither graft fracture nor lysis was observed. Eleven patients underwent surgical revision, with nine of these revisions related to the reconstruction. The average Musculoskeletal Tumor Society score at the time of the latest follow-up was 73% of the maximal possible score. The average score was 82% for the eleven patients with an age of less than twenty years at the time of the index procedure and 65% for the thirteen older patients. Ten patients walked without any assistive device, and five of them had normal function with no or only a slight limp. CONCLUSIONS: Pelvic reconstruction after a limb-sparing resection is associated with a high risk of surgical complications and usually should be reserved for patients with a primary bone sarcoma. A pelvic allograft can restore the anatomy and provide good functional results, especially in young patients. Nonunion was the most common allograft-related complication.     

Cutaneous malignant melanoma in the young

Cutaneous malignant melanoma in children and adolescents is rare. Of over 1600 patients documented in the Melanoma Registry at Frenchay Hospital from 1967 onwards, only 29 cases are recorded who were 21 years of age or younger. Of these, four patients were pre-pubertal (13 years or less) and none arose from a congenital "giant" naevus. Fifteen patients developed metastases, 10 of whom showed spread of tumour within 30 months of initial presentation. Eight of these patients with metastatic disease died, with a maximum survival of 61 months. Only seven patients, five of whom have metastatic disease, survive more than 10 years after first presentation; nine patients, one of whom has secondaries, survive for 5 years or less.     

Primary mucosal malignant melanoma of the oral cavity

Mucosal malignant melanoma arising from the head and neck region is a rare entity, and it is more aggressive than cutaneous melanoma. Furthermore, the complex anatomy of the oral cavity makes complete surgical excision difficult. Thus, early diagnosis and treatment of a mucosal lesion are important. In this study, three mucosal malignant melanoma cases and the literature have been reviewed. Three cases who presented with a primary malignant melanoma of the oral cavity have been retrospectively analyzed. All three patients were female with a mean age of 31.3 years, and the median follow-up period was 18.6 (6–36) months. The tumor was located on the maxillary gingiva in case 1 and in the hard palate–maxillary gingiva in cases 2 and 3. Case 2 had a distant metastasis during first admission. The tumor was excised with a 2-cm surgical margin in all cases. Case 2 received adjuvant chemotherapy. During the follow-up period, case 1 had a cervical lymphadenopathy 8 months after the first operation, so she underwent cervical lymph node dissection then received chemotherapy. Melanoma of the oral cavity is very rare with an extremely poor prognosis. As some melanomas may be amelanotic, a high index of suspicion is necessary. A biopsy should be taken from any suspicious lesion in the oral cavity. Surgical excision combined with adjuvant therapy is the main treatment approach for these cases. Prognosis of the disease depends on early diagnosis and treatment. A multicenter prospective study is required to introduce staging of the disease and the optimal treatment regimen.