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[Atypical sarcomas localized within a head and neck in children].

Seven children treated surgically because of non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS) localized extrameningeally on the head and neck were presented. Three of the patients were operated on haemangiopericytoma, two--fibrosarcoma, one child--neurofibrosarcoma and one--liposarcoma. The pre- and postoperative TNM classification was employed as a staging system. The surgical resectability--R was used to establish tumour margins. Four patients (two with fibrosarcoma and two with haemangiopericytoma) survived free of disease. The influence of the complete surgical resection on the outcome of the head and neck extrameningeal NRSTS was proved.

Child↗

[Von Recklinghausen's neurofibromatosis (neurofibromatosis type I)--a familial case report].

INTRODUCTION: Neurofibromatosis is a term used for two disorders: NF-1 and NF-2. NF-1 is Von Recklinghasusen's neurofibromatosis and comprises characteristic skin lesions (cafe au lait spots, intertriginous freckles, neurofibromatous skin tumors) and other congenital and hamartomatous bone, endocrine glands and central nervous system lesions. Its incidence is one in every 2500 to 3300 births. CASE REPORT: Two female patients, a 20 years old daughter and her mother 46 years of age were admitted to the Clinic of Dermatovenereology in Novi Sad due to appearance of many sessile and pedunculated neurofibromas, cafe au lait spots and freckles on their trunks, axillary and inguinal regions. Laboratory findings showed no abnormalities. Both of them were examined by many specialists. No systemic disturbances were established. The daughter was sent to plastic surgery for operative treatment. DISCUSSION: The clinical presentation of NF-1 is very variegated. Beside characteristic skin lesions, other clinical features include skeletal bony abnormalities, mental deficiency, seizures, neurofibromas of the spinal and cranial nerve roots, iris hamartomas, optic nerve gliomas, endocrine disorders, endocrine tissue tumors, other visceral tumors, etc. Some of these disorders can be life-threatening. Malignant transformations of the NF-1 lesions occur approximately in 5% of patients, most often as neurofibrosarcomas, Wilms' tumors, rhabdomyosarcomas or various forms of leukemias. CONCLUSION: We present familial cases of Von Recklingausen's neurofibromatosis without systemic abnormalities so far. The clinical course of this disease is unpredictable and a multidiscipline clinical assessment is necessary during whole life.

Adult↗

[Clinicopathological characteristics and surgical treatment of primary neurogenic tumors of the mediastinum].

OBJECTIVE: To study the clinical and histopathological characteristics, and surgical treatment of primary neurogenic tumors of the mediastinum. METHODS: The experience in diagnosis and treatment of 125 patients with primary neurogenic tumors of the mediastinum from Jan. 1965 to Dec. 1998 in our hospital was summarized. All patients were operated and pathologically confirmed. RESULTS: Primary neurogenic tumor accounted for 125(22.0%) of 568 patients with primary tumors of the mediastinum in the same period. The incidence of neurilemoma was 48.9% (61/125), neurofibroma 27.2% (34/125), ganglioneuroma 13.6% (17/125), and other rare tumors 10.4% (13/125, neurofibrosarcoma 4, ganglioneuroblastoma 3, chemodectoma 3, pheochromocytoma 2, and neuroblastoma 1). In this series, 78 patients did not have any subjective symptoms, and the tumors were found on routine physical checkup. In 117 patients, the tumors were located in the posterior mediastinum. In all bwt 2 patients, the tumor was resectable. The postoperative complication rate was 4.0% and operative mortality rate was 0.8%. The 3-year survival rate in patients with malignant tumors was 18.2%. CONCLUSION: The clinical characteristics and prognosis of primary neurogenic mediastinal tumors varies with their histopathology. Surgical operation is the first choice of treatment.

Adolescent↗

[Association of nm23-H1 with orthopedic oncological conditions].

OBJECTIVE: To explore the correlation between nm23-H1 gene and malignant and semi-malignant bone tumors. METHOD: Thirty surgical specimens were collected, including 8 osteosarcomas, 7 giant cell tumors, 4 chondrosarcomas, 2 Ewing's sarcomas, 2 malignant fibrohistocytomas, 1 neurofibrosarcoma, 1 schwannoma, and 5 metastatic cancers. The expression of nm23-H1 in these specimens was observed by in situ hybridization and immunohistochemical staining. RESULT: The expression of nm23-H1 in 4 cases of primary giant cell tumor (GCT) was high, but was low or absent in 3 recurrent ones. In 4 cases of chondrosarcomas, the expression was positive. Its expression was basically negative in Ewing's tumor, malignant fibrous histiocytoma and metastatic sarcomas. CONCLUSION: There is no association of nm23-H1 with osteosarcoma.

Adult↗

[G-CSF and GM-CSF in treatment of neutropaenia after chemotherapy in children with neoplasms].

A total number of 608 cycles of G-CSF and/or GM-CSF was applied in 280 patients aged from 6 months to 20 years during neutropaenia associated with chemotherapy of children's neoplasms (NHL-124, NBL-42, RMS-36, Nephroblastoma-18, Osteosarcoma-17, Ewing's Sarcoma-14, Hepatoblastoma-6, Neurofibrosarcoma-6, PNET-5, Medulloblastoma-3, Fibrohistiocytoma-3, Angiosarcoma-2, other - 4). G-CSF - Neupogen (Filgastrim, Hoffman La Roche - 492 cycles) and GM-CSF - Leucomax (Molgramostim, Shering Plough - 116 cycles) were administered 5 mg/kg/day s.c. Forty one children with malignancies (NHL -21 cases, solid tumours -17) treated before cytokines were in use served as a control group. Our study demonstrated that G-CSF and GM-CSF therapy, gives a shorter period of neutropaenia, reduction of the number of febrile days, decreased frequency of infection and shortened its duration.

Adolescent↗

[Recurrent benign tumors of parotid gland: the role of the surgery].

Parotid gland tumor recurrences can prove problematic both in terms of facial nerve trauma during surgery and for the possible transformation into malignancy. Between 1981 and 2001 a total of 40 patients (23 women, 17 men; average age 48; age range 20-79 years) underwent surgery for recurrent parotid gland tumors. The average time between the first and the second surgical procedures was approximately 10 years. Five subjects underwent surgery several times for the same pathology. The Authors feel that MRI imaging is an essential tool for the evaluation of infiltrations into the soft tissues. The following surgical procedures were performed: enucleation in 2 cases; exofacial partial parotidectomy (PP) in 1; lower polar PP with functional neck dissection in 1; total parotidectomy (TP) with preservation of the facial nerve in 31 cases; TP with functional neck dissection in 2; TP with sectioning of the facial nerve in 2 and TP with transmandibular buccopharyngectomy and myocutaneous gran dorsal muscle flap in 1 case. Histology proved positive for the following: pleomorphous adenoma in 21 cases, adenocarcinoma in 11, aggressive fibromatosis in 2, cystadenolymphoma in 1, parotiditis in 1, lipoma in 1, cystic lymphoid hyperplasia in 1, histiofibrosarcoma in 1 and neurofibrosarcoma in 1. The surgical technique used was retrograde dissection of the facial nerve starting from one of the peripheral branches. Post-operatively, whenever a facial paralysis was encountered it proved difficult to recover. Paralysis of the nerve was permanent only in the 2 subjects where sectioning proved necessary because of infiltration by carcinoma. In 6 subjects recovery of the paralysis took one year, in 4 it took 6 months and in 2 other cases 3 months. In 22 cases there was only a slight paralysis of some branches which recovered during the post-operative period. We do not have definitive data on 4 subjects either because the period of time since surgery is still too short or because they did not come in for subsequent check-ups. The results of our study show that total parotidectomy should be the treatment of choice in case of benign parotid gland tumors and in particular for pleomorphic adenoma.

Adult↗

[Diagnosis and surgical management of mediastinal neurogenic tumors].

OBJECTIVE: To study the clinical manifestations, diagnostic methods, surgical management and prognosis of patients with neurogenic tumors of the mediastinum. METHOD: One hundred and ten patients with neurogenic tumors of the mediastinum were analyzed retrospectively. RESULTS: After operation, 2 patients died in hospitalization and 8 experienced such complications as Horner's syndrome or laryngeal recurrent nerve paralysis. In 102 patients with benign tumors, 2 patients had recurrence, and 4 patients with neurofibrosarcoma or malignant neurilemmoma died within 3 years postoperatively. CONCLUSIONS: Most neurogenic tumors of the mediastinum are benign and could be diagnosed by chest X-ray or CT. The clinical manifestations, diagnosis methods, surgical management of the dumbbell tumors differ from others. Minimal invasive surgery and video assist thoracoscopy surgery are of special value in treatment of the selected neurogenic tumors of the mediastinum. Benign neurogenic tumors rarely recur after complete resection, and malignant neurogenic tumors have poor prognosis.

Adolescent↗

Atypical fibroxanthoma of the skin. A clinico-pathological study of 57 cases.

A retrospective study of 57 patients with atypical fibroxanthoma of the skin is presented. The light microscopy is described and the differential diagnosis is discussed. Most of the atypical fibroxanthomas (32 out of 57 cases) were originally diagnosed as soft tissue sarcomas, e.g. fibrosarcoma, dermatofibrosarcoma, neurofibrosarcoma, myosarcoma and unspecified sarcoma. The tumour occurred chiefly in middle-aged and elderly patients; three patients were 15 years old or younger. The median age was 73 years in patients in whom the tumour occurred in the head and neck, and 34 years in patients in whom the tumour developed on the extremities and trunk. The sex ratio (male to female) was almost equal. Follow-up information about 43 patients was available. The follow-up period ranged from 1 year to 25 years with a median of 9 years. Eight patients died from intercurrent disease; all the other 35 patients are alive and well. The clinical course was benign in all but one patient in whom a recurrence developed and metastases to the regional lymph nodes appeared 7 years after the initial excision. The tumour in this case did not differ histologically from the other atypical fibroxanthomas with respect to cellularity, cellular and nuclear atypia or mitotic activity. It is suggested that the recurrence per se might be of prognostic importance.

Adolescent↗

NTP Toxicology and Carcinogenesis Studies of Isophorone (CAS No. 78-59-1) in F344/N Rats and B6C3F1 Mice (Gavage Studies).

Toxicology and carcinogenesis studies of isophorone (greater than 94% pure), a widely used solvent and chemical intermediate, were conducted by administering 0, 250, or 500 mg isophorone/kg body weight per day by gavage in corn oil to groups of 50 F344/N rats and 50 B6C3F1 mice of each sex, 5 days per week for 103 weeks. Doses selected for the 2-year studies were based on the 16-day studies in which rats and mice of each sex received doses of 0-2,000 mg/kg per day and on 13-week studies in which rats and mice of each sex received doses ranging from 0 to 1,000 mg/kg per day by gavage in corn oil. No chemically related gross or histopathologic effects were observed in the 16-day or 13-week studies, but 1/5 high dose male rats, 4/5 high dose female rats, and all high dose male and female mice died during the 16-day studies. During the 13-week studies, 1/10 high dose female rats and 3/10 high dose female mice died. The high dose for the 2-year studies was set at 500 mg/kg per day for each sex of rats and mice, based mainly on the deaths in the 13-week studies. Throughout the 2-year study, the mean body weights of the high dose male rats averaged 5% lower than those of the vehicle controls. During the second year, the mean body weights of the female high dose rats averaged 8% lower than those of the vehicle controls, and the high dose female mice averaged 5% lower. The survival of high dose male rats was significantly lower than that of the vehicle controls after week 96 (final survival: vehicle control, 33/50; low dose, 33/50; high dose, 14/50). The survival of dosed female rats was poor (30/50; 23/50; 20/50), due in part to 20 gavage-related accidental deaths of dosed animals. The survival of male mice was also low (16/50; 16/50; 19/50), but there was a significant trend toward increased survival of dosed female mice relative to that of the vehicle controls (26/50; 35/50; 34/50). Dosed male rats showed a variety of proliferative lesions of the kidney (tubular cell hyperplasia: 0/50; 1/50; 4/50; tubular cell adenoma: 0/50; 0/50; 2/50; tubular cell adenocarcinoma: 0/50; 3/50; 1/50; epithelial hyperplasia of the renal pelvis: 0/50; 5/50; 5/50). Dosed male rats also exhibited increased mineralization of the medullary collecting ducts (1/50; 31/50; 20/50), and low dose male rats showed a more severe nephropathy than is commonly seen in aging F344/N rats. Carcinomas of the preputial gland were increased in high dose male rats (0/50; 5/50; 5/50). With the exception of a moderate increase in nephropathy (21/50; 39/50; 32/50), female rats did not show chemically related increased incidences of neoplastic or nonneoplastic lesions. In high dose male mice, isophorone exposure was associated with increased incidences of hepatocellular adenomas and carcinomas (18/48; 18/50; 29/50) and of mesenchymal tumors of the integumentary system (fibroma, fibrosarcoma, neurofibrosarcoma, or sarcoma: 6/48; 8/50; 14/50). An increased incidence of lymphomas or leukemias was noted in low dose male mice (8/48; 18/50; 5/50). Coagulative necrosis (3/48; 10/50; 11/50) and hepatocytomegaly (23/48; 39/50; 37/50) were observed more frequently in the livers of dosed male mice than in vehicle controls. No compound-related neoplastic or nonneoplastic lesions associated with isophorone exposure were seen in female mice. Isophorone was not mutagenic in strains TA100, TA1535, TA1537, or TA98 of Salmonella typhimurium in the presence or absence of Aroclor 1254-induced male Sprague-Dawley rat or male Syrian hamster liver S9. Isophorone was weakly mutagenic in the mouse L5178Y/TK+/- assay in the absence of S9; it was not tested in the presence of S9. Isophorone induced sister-chromatid exchanges in the absence of S9 in Chinese hamster ovary cells; it did not induce sister-chromatid exchanges in the presence of Aroclor 1254-induced male rat liver S9, and it did not induce chromosomal aberrations in Chinese hamster ovary cells in the presence or absence of S9. An audit of the experimental data was conducted for the 2-year toxicology and carcinogenesis studies of isophorcarcinogenesis studies of isophorone. No data discrepancies were found that influenced the final interpretations. Under the conditions of these 2-year gavage studies, there was some evidence of carcinogenicity of isophorone in male F344/N rats as shown by the occurrence of renal tubular cell adenomas and adenocarcinomas in animals given 250 or 500 mg/kg per day; carcinomas of the preputial gland were also observed at increased incidence in male rats given 500 mg/kg. There was no evidence of carcinogenicity in female F344/N rats given 250 or 500 mg/kg per day. For male B6C3F1 mice, there was equivocal evidence of carcinogenicity of isophorone as shown by an increased incidence of hepatocellular adenomas or carcinomas (combined) and of mesenchymal tumors in the integumentary system in animals given 500 mg/kg per day and by an increase in malignant lymphomas in animals given 250 mg/kg per day. There was no evidence of carcinogenicity of isophorone in female B6C3F1 mice given 250 or 500 mg/kg per day. Synonym: 3,5,5-trimethyl-2-cyclohexen-1-one

Journal Article↗

NTP Toxicology and Carcinogenesis Studies of Chlorendic Acid (CAS No. 115-28-6) in F344/N Rats and B6C3F1 Mice (Feed Studies).

Chlorendic acid is a chemical intermediate used in the preparation of fire-retardant polyester resins and plasticizers. Toxicology and carcinogenesis studies of chlorendic acid (greater than 98% pure) were conducted by administering the chemical in feed to groups of 50 male and 50 female F344/N rats and B6C3F1 mice at concentrations of 0, 620, or 1,250 ppm for 103 weeks. The estimated mean daily consumption of chlorendic acid was 27 and 56 mg/kg body weight for low dose and high dose male rats and 39 and 66 mg/kg for low dose and high dose female rats. In mice, the estimated daily consumption was 89 and 185 mg/kg for low dose and high dose males and 100 and 207 mg/kg for low dose and high dose females. These concentrations were selected because higher levels in the 14-day and 13-week studies caused decreased mean body weights, more deaths, and increased incidences of liver lesions (rats: centrilobular cytomegaly, mitotic alterations, bile duct hyperplasia; mice: centrilobular cytomegaly, mitotic alterations, coagulative necrosis) relative to control groups. Survival and feed consumption of dosed male and female rats and mice in the 2-year studies were similar to those of controls. Mean body weights of high dose male and female rats and mice were lower than those of controls. Mean body weights of high dose female rats were 16%-24% lower than those of controls during the second half of the study. In the 2-year chlorendic acid feed studies, incidences of nonneoplastic lesions of the liver in dosed male rats (cystic degeneration) and dosed female rats (granulomatous inflammation, pigmentation, and bile duct hyperplasia) were increased. The incidences of neoplastic nodules of the liver were significantly increased in dosed male rats (control, 2/50; low dose, 21/50; high dose, 23/50) and high dose female rats (1/50; 3/39; 11/50). The incidence of hepatocellular carcinomas was also increased in high dose female rats (0/50; 3/49; 5/50). In mice, the incidences of nonneoplastic lesions of the liver were increased in dosed males (coagulative necrosis) and high dose females (mitotic alterations). The incidences of hepatocellular adenomas (5/50; 9/49; 10/50), hepatocellular carcinomas (9/50; 17/50; 20/50), and hepatocellular adenomas or carcinomas (combined) (13/50; 23/49; 27/50) were increased in dosed male mice. Hepatocellular carcinomas metastasized to the lung in 2/50 control, 4/49 low dose, and 7/50 high dose male mice. Hepatocellular adenomas or carcinomas (combined) were not significantly increased in female mice (3/50; 7/49; 7/50). The incidences of acinar cell hyperplasia (0/49; 4/50; 4/50) and acinar cell adenomas (0/49; 4/50; 6/50) of the pancreas were increased in dosed male rats relative to those of controls. Pancreatic acinar cell adenoma is an uncommonneoplasm in untreated control F344/N rats in NTP studies (3/1,667). In dosed male rats, incidences of alveolar/bronchiolar adenomas of the lung (0/50; 3/50; 5/50) were increased. The incidences of alveolar/bronchiolar adenomas or carcinomas (combined) in dosed female mice were also increased (1/50; 5/50; 6/50). Preputial gland carcinomas occurred at a greater incidence in low dose male rats (1/50; 8/50; 4/50) than in controls. An adenoma and a squamous cell papilloma were observed in two low dose male rats. The incidences of sarcomas, fibrosarcomas, or neurofibrosarcomas (combined) of the salivary gland (1/50; 2/49; 4/50) were increased in dosed male rats. The incidences in the dosed groups were not significantly different from that in the controls, but these tumors are uncommon in F344/N rats receiving no treatment (3/1,689). Chlorendic acid was not mutagenic in strains TA100, TA98, TA1535, or TA 1537 of Salmonella typhimurium in the presence or absence of Aroclor 1254-induced male Sprague-Dawley rat or male Syrian hamster liver activation when tested according to the preincubational protocol. Chlorendic acid was mutagenic in the L5178Y/TK+/- mouse lymphoma cell forward assay (in the absence of activation) at a dose resulting in toxicity. An audit of the experimental dudit of the experimental data was conducted for the 2-year studies of chlorendic acid. No data discrepancies were found that influenced the final interpretations. Under the conditions of these 2-year feed studies, there was clear evidence of carcinogenicity of chlorendic acid for male F344/N rats as shown by increased incidences of neoplastic nodules of the liver and acinar cell adenomas of the pancreas. Increased incidences of alveolar/bronchiolar adenomas and preputial gland carcinomas may also have been related to the administration of chlorendic acid. There was clear evidence of carcinogenicity of chlorendic acid for female F344/N rats as shown by increased incidences of neoplastic nodules and of carcinomas of the liver. There was clear evidence of carcinogenicity of chlorendic acid for male B6C3F1 mice as shown by increased incidences of hepatocellular adenomas and of hepatocellular carcinomas. There was no evidence of carcinogenicity of chlorendic acid for female B6C3F1 mice given chlorendic acid in the diet at concentrations of 620 or 1,250 ppm for 103 weeks.

Journal Article↗

NTP Toxicology and Carcinogenesis Studies of Boric Acid (CAS No. 10043-35-3) in B6C3F1 Mice (Feed Studies).

Boric acid is a component of cosmetics and pharmaceuticals and is also used in numerous industrial processes. Earlier long-term studies did not demonstrate a carcinogenic effect in Sprague-Dawley rats. Because of potential widespread human exposure, corroborative evidence was sought in a second species. Toxicology and carcinogenesis studies were conducted by feeding technical-grade boric acid (99.7% pure) to groups of male and female B6C3F1 mice for 14 days, 13 weeks, and 2 years. In the 14-day studies (five mice per group), mortality occurred in mice fed 25,000 ppm, 50,000 ppm, or 100,000 ppm boric acid; hyperplasia and/or dysplasia of the forestomach was also seen in these dose groups. No compound-related gross pathologic or histopathologic effects were seen in male or female mice exposed at concentrations up to 12,500 ppm in feed. In the 13-week studies, groups of 10 male and 10 female mice were fed boric acid at concentrations up to 20,000 ppm; 8 male mice and 1 female mouse receiving 20,000 ppm and 1 male receiving 10,000 ppm boric acid died before the end of the studies. Male and female mice receiving 20,000 ppm boric acid weighed 23% and 18% less, respectively, than did the controls at the end of the studies. Testicular atrophy in 8/10 male mice, hyperkeratosis and acanthosis of the stomach in 8/10 male and female mice, and extramedullary hematopoiesis of the spleen in all male and female mice receiving 20,000 ppm boric acid indicated that the testis, stomach, and spleen were potential target organs in the 2-year studies. Based on these results, 2-year toxicology and carcinogenesis studies were conducted by feeding diets containing boric acid at concentrations of 0, 2,500, or 5,000 ppm to groups of 50 male and 50 female mice. Survival of high dose male mice after week 63 and of low dose mice after week 84 was lower than that of the controls (final survival: control, 41; low dose, 30; high dose, 22), which may have reduced the sensitivity of the carcinogenicity study; the numbers of female mice (33; 33; 37) that survived to the end of the studies were considered adequate for toxicologic evaluation. Body weight gain was reduced in each sex after week 30; mean final body weights were 7% and 13% below control values for exposed male mice and 7% and 20% below those of controls for exposed female mice. No chemically related clinical signs were reported. At the top dose, boric acid caused an increased incidence of testicular atrophy (control, 3/49; low dose, 6/50; high dose, 27/47) and interstitial cell hyperplasia (0/49; 0/50; 7/47) inmale mice. The testicular atrophy was characterized by variable loss of spermatogonia, primary and secondary spermatocytes, spermatids, and spermatozoa from the seminiferous tubules. The seminiferous tubules contained primarily Sertoli cells and variable numbers of spermatogonia. In some mice, there were accumulations of interstitial cells, indicating hyperplasia. In low dose male mice, there were increased incidences of hepatocellular carcinomas (5/50; 12/50; 8/49) and hepatocellular adenomas or carcinomas (combined) (14/50; 19/50; 15/49) and an increased incidence of subcutaneous tissue fibromas, sarcomas, fibrosarcomas, or neurofibrosarcomas (combined) (2/50; 10/50; 2/50). No increased incidence of subcutaneous tissue neoplasms was seen in male mice receiving 5,000 ppm. Because the incidence of subcutaneous tissue tumors is variable in historical controls, because there was no corresponding increase in the high dose male mice, and because the incidence of hepatocellular tumors was not significant by the incidental tumor test and was within the historical control range, neither of these tumors was considered to be related to the administration of boric acid. Boric acid was not mutagenic in the Salmonella/microsome assay with Salmonella typhimurium strains TA98, TA100, TA1535, or TA1537. Boric acid was negative in the mouse lymphoma L5178Y/TK+/- assay and did not induce sister-chromatid exchanges or chromosomal aberrations in Chinese hamster ovary cells. All assays were preformed with anhamster ovary cells. All assays were preformed with and without metabolic activation. The data, documents, and pathology materials from the 2-year studies of boric acid were audited at the NTP Archives. The audit findings show that the conduct of the studies is documented adequately and support the data and results given in this Technical Report. Under the conditions of these 2--year feed studies, there was no evidence of carcinogenicity of boric acid at doses of 2,500 or 5,000 ppm for male or female B6C3F1 mice. Testicular atrophy and interstitial cell hyperplasia were observed in high dose male mice. The decrease in survival of dosed male mice may have reduced the sensitivity of this study. Synonyms: orthoboric acid; boracic acid

Journal Article↗

[The microsurgery of the tumor involving Meckel's cave and nearby area].

A retrospective review of 12 cases of tumor involving Mackel's cave and nearby area seen at the Tongji hospital in a 20-year period. All of the cases may be divided to three clearly defined clinical groups: (1) Patients with typical trigeminal neuralgia. The tumor usually involve the ganglion. After removal the mass the prognosis were excellent. (2) Schwannoma or neurofibrosarcoma embedded in the semilunar ganglion with a history of atypical face pain without neurological deficit. After operation the pain relief was not as good as the group 1. (3) Patients with the history of face dysethesias and pain, tumors usually involving the middle fossa of cranial base with multiple cranial nerve deficit. These patients had variable mass lesions and after removed tumor the prognosis was not as good as in group 1 and group 2. The anatomy of Meckel's cave and surgical technique in treating these tumors were discussed also.

Adolescent↗

Overexpression of the c-erbB-2 oncogene in sarcomas and small round-cell tumors of childhood. An immunohistochemical investigation.

Amplification and overexpression of the c-erbB-2 oncogene have been associated with a poor prognosis in breast cancer. The present study evaluates c-erbB-2 protein over-production in sarcomas and in small round-cell tumors of childhood by applying an immunohistochemical technique to formalin-fixed, paraffin-embedded tissues. This technique had previously demonstrated high sensitivity for the detection of c-erbB-2 activation. One peripheral neuroepithelioma demonstrated equivocal immunoreactivity for c-erbB-2 protein. There was no evidence of c-erbB-2 protein overproduction in 11 angiosarcomas, 21 chondrosarcomas, 10 epithelioid sarcomas, 14 Ewing's sarcomas, 20 hepatoblastomas, nine leiomyosarcomas, 12 liposarcomas, 13 malignant fibrous histiocytomas, nine malignant lymphomas, 15 neuroblastomas/ganglioneuroblastomas, 16 neurofibrosarcomas, 10 osteosarcomas, seven peripheral neuroepitheliomas, 10 rhabdomyosarcomas, six synovial sarcomas, and 20 Wilm's tumors. We conclude that overproduction of the c-erbB-2 protein is rare or absent in these neoplasms and that its detection is therefore not likely to be of practical utility in their pathologic evaluation and prognostication.

Bone Neoplasms↗

Soft tissue tumors in Ramathibodi Hospital: study of 1,682 cases during 1977-1986.

A series of 1,682 cases in the service of the Department of Pathology, Ramathibodi Hospital from 1977 to 1986 was studied and classified according to WHO histologic typing of soft tissue tumors. The findings revealed that the three most common benign soft tissue tumors were lipoma (50.6%), vascular tumors (17.4%) and neurofibroma (7.3%), whereas, the three most common malignant soft tissue tumors were unclassified sarcoma (16.5%), rhabdomyosarcoma (15.5%) and neurofibrosarcoma (10.7%). Lipoma occurred at any age group with a mean age of 40 years and affected males and females equally and common sites of involvement were neck (19.7%), back (19.7%) and upper arm (18.4%). Unclassified sarcoma was the most common among the malignant group accounting for 16.5 per cent of the total malignant soft tissue tumors. The age ranged from 7 to 70 years with a mean age of 27.4 years. The most common sites of involvement were the extremities (50%) especially the thigh.

Adolescent↗

Osteosarcoma as a second malignant neoplasm in children.

Nine patients who had an osteosarcoma that had developed as a second malignant neoplasm in a previously irradiated site were managed at a major center for the treatment of tumors in children. The doses of radiation had averaged 4144 centigray (range, 2300 to 8000 centigray) and chemotherapy had been administered, when appropriate, for the primary malignant lesion (Ewing sarcoma, malignant fibrous histiocytoma, Hodgkin lymphoma, neuroblastoma, neurofibrosarcoma, rhabdomyosarcoma, and Wilms tumor). The interval between the initial treatment and the diagnosis of the secondary sarcoma averaged ten years and one month (range, five years and ten months to twenty-one years and nine months). Three patients were alive, two of them with active disease, at the time of writing. The other six had died within three years (average, fifteen months) after the second diagnosis. The prevalence of secondary osteosarcoma is increasing as the survival of children who have a malignant lesion increases. Plans for tumor therapy should take into account the risk of this complication, which is usually fatal.

Adolescent↗

[A rare cardiac tumor: the malignant fibrous histiocytoma. Description of a case].

The present report describes the case of a 61-year-old woman with malignant fibrous histiocytoma of the left atrium originating from the left atrial free wall, operated on in emergency for a suspected large left atrial myxoma that, at the echo scan, was consistently protruding through the left atrioventricular orifice at each diastole and was almost completely occluding the left ventricular inflow, causing signs of congestive heart failure and severe dyspnea. Surgery was performed as radically as possible, but the histological examination of the specimen revealed the exact diagnosis of the neoplasm. About 75% of primary tumors are benign and 75% of these are atrial myxomas. The malignant tumors consist of various sarcomas: myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, undifferentiated sarcoma, reticulum cell sarcoma, neurofibrosarcoma, and malignant fibrous histiocytoma. The long-term results for sarcomas are very poor and there are few survivors after several months from surgery due to the extent of local spread and invasion or because of the frequent distant metastases. Malignant fibrous histiocytoma constitutes about 2% of all cardiac malignancies, which might grow within several localized areas, occasionally in the heart. Echocardiography represents the best examination procedure for both diagnosis and follow-up of patients with cardiac tumors.

Female↗

Induced soft tissue sarcomas following radiation treatment for uterine carcinomas.

Twenty-two cases of sarcomas arising from irradiated soft tissues were observed in 531 patients operated on for soft tissue sarcomas over 38 years in our Department. Eleven women were initially irradiated for cervical cancer (ce-ca), two for endometrial cancers. The remaining nine patients were irradiated for other lesions, malignant or benign. The median interval between the radiation for uterine cancer and the outcome of clinical symptoms of a sarcoma was 18 years. Sarcomas more frequently arose in the posterior fields (buttock or sacral region in nine patients) than in the anterior (pubis, hypogastrium-four patients). The infiltration of skin was almost always present (10/13), in seven cases with an ulceration (7/13). Histologically, four hemangiosarcomas, four fibrosarcomas, three malignant fibrous histiocytomas and two neurofibrosarcomas were found. The irradiation administered to ce-ca patients consisted of radium (22.0 mcd to 58.0 mcd) and an external radiation from X-ray units (except in one case) from two pairs of two anterior and posterior fields, with skin doses of 3200 cGy to 4200 cGy directly on one field plus several hundreds cGy from the opposite field. Three-year overall survival rate was low (30%) similar to that observed in patients with primary high-grade (G-3) sarcomas. We observed more cases subjected to radiotherapy for cervical cancer than might be expected.

Adult↗

HLA-DR (Ia-like) reactivity in tumors of bone and soft tissue: an immunohistochemical comparison of monoclonal antibodies LN3 and LK8D3 in routinely processed specimens.

Recent studies of Class II histocompatibility antigen expression in bone and soft tissue sarcomas have suggested that malignant fibrous histiocytoma (MFH) may express HLA-DR, whereas histologically similar pleomorphic, epithelioid, and spindle cell malignant neoplasms generally do not. To test whether these observations are reproducible in the differential diagnosis of soft tissue sarcomas, anti-HLA-DR antibodies LK8D3 and LN3 were applied to formalin-fixed, paraffin-embedded sections of MFH, neurofibrosarcoma (NFS), leiomyosarcoma (LMS), synovial sarcoma (SS), fibrosarcoma (FS), angiosarcoma (AS), Kaposi's sarcoma (KS), chondrosarcoma (ChS), "dedifferentiated" chondrosarcoma (DChS), osteosarcoma (OS), epithelioid sarcoma (ES), and clear cell sarcoma (CCS; malignant melanoma of soft parts). The only consistent difference in Class II antigen expression was seen in the group of neoplasms composed of large polygonal cells. Among the latter lesions, four of six clear cell sarcomas were labeled by LK8D3 or LN3, but none of 12 epithelioid sarcomas were reactive. Otherwise, a diversity of tumors in other morphologic categories expressed Class II antigens, with no clear diagnostic patterns. These results may be of use in the diagnostic separation of large cell epithelioid tumors of soft tissue, but neither LN3 nor LK8D3 appears to be helpful in the identification of other sarcomas.

Antibodies, Monoclonal↗