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Background: Survivors of hereditary retinoblastoma have an increased risk for second malignancies, especially soft tissue sarcomas. However, the risks of individual histologic subtypes of soft tissue sarcomas have not been evaluated., Methods: We estimated the risk for six subtypes of soft tissue sarcomas (fibrosarcoma, liposarcoma, histiocytoma, leiomyosarcoma, rhabdomyosarcoma, and others) in a cohort of 963 one-year survivors of hereditary retinoblastoma among patients diagnosed at two US institutions from 1914 through 1984. We calculated standardized incidence ratios (SIRs) for specific subtypes of soft tissue sarcomas by comparison with population data from the Connecticut Tumor Registry or from National Cancer Institute Surveillance, Epidemiology, and End Results database. We also calculated the cumulative risk for all soft tissue sarcomas combined., Results: We observed 69 soft tissue sarcomas in 68 patients with hereditary retinoblastoma. Risks were elevated for soft tissue sarcomas overall (SIR = 184, 95% confidence interval [CI] = 143 to 233) and for individual subtypes. Leiomyosarcoma was the most frequent subtype (SIR = 390, 95% CI = 247 to 585), with 78% of leiomyosarcomas diagnosed 30 or more years after the retinoblastoma diagnosis (SIR = 435, 95% CI = 258 to 687). Among patients treated with radiotherapy for retinoblastoma, we found statistically significantly increased risks of soft tissue sarcomas in the field of radiation. Irradiated patients also had increased risks of soft tissue sarcomas, especially leiomyosarcomas, outside the field of radiation, and risks of soft tissue sarcomas were increased in nonirradiated patients as well, indicating a genetic predisposition to soft tissue sarcomas independent of radiation. The cumulative risk for any soft tissue sarcoma 50 years after radiotherapy for retinoblastoma was 13.1% (95% CI = 9.7% to 17.0%)., Conclusion: Long-term follow-up of a cohort of survivors of hereditary retinoblastoma revealed a statistically significant excess of leiomyosarcoma and other soft tissue sarcomas that persists decades after the retinoblastoma diagnosis. Retinoblastoma survivors should undergo regular medical surveillance for sarcomas in their adult years.

The acceleration of carcinogenesis, which was induced either by radio frequency radiation from a cellular telephone or by the ferric-ATP complex, was similar in a mouse strain characterized by age-determined carcinogenesis of lymphoid tissues. Organ hypertrophy, the presence of lymphoid blood and ascites, the development of solid tumours, and mortality were very different to those found in control animals. These results emphasize the role of calcium ion signal influx in the activation of oncogenes and the failure of thymus-determined immune defences.

This case describes a twin with bilateral retinoblastoma who developed leiomyosarcoma of the bladder at age 17 and again at 39. At 17-years of age she was diagnosed with a leiomyosarcoma of the bladder after presenting with recurrent urinary tract infections, hematuria, and dysuria. She was treated with partial cystectomy. After a 12-year disease-free interval, she was diagnosed with a second leiomyosarcoma of the bladder. This case supports the relationship between the genetic form of retinoblastoma and leiomyosarcoma and illustrates the necessity for extensive follow-up and well-defined treatment of secondary neoplasms., ((c) 2006 Wiley-Liss, Inc.)

This is the first report of a myxoid leiomyosarcoma arising in a cirrhotic liver. The tumor was resected from a 64-year-old man. On gross examination, it was soft and hemorrhagic. The tumor was composed of deceptively benign-looking smooth muscle cells with clear cytoplasm suspended in a myxoid stroma with foci of hemorrhage. Immunohistochemistry and electron microscopy confirmed that this was a smooth muscle cell neoplasm. The abundance of glycogen and ultrastructural signs of smooth muscle differentiation were considered consistent with an immature smooth muscle cell phenotype consistent with the diagnosis of myxoid leiomyosarcoma. Since myxoid leiomyosarcomas are aggressive tumors, it is important to recognize them histologically and also bear in mind that these tumors can occur even in unusual extrauterine locations such as a cirrhotic liver.

We report the case of a young girl treated at age 16 for a progressive scoliosis by posterior instrumented arthrodesis. Ten years later, she suddenly developed lumbar pain and paraplegia. The surgical procedure showed a mass infiltrating the vertebral canal and the dural sheath following a supralaminar hook. Histology revealed a diagnosis of leiomyosarcoma. The outcome was poor with a rapid and fatal extension of the tumor. There is nothing to justify a causal link between the scoliosis and the late onset of a malignant tumor. Nevertheless, we discuss the potential role of diagnostic irradiation consecutive to scoliosis monitoring during growth and the potential role of environmental carcinogens like metallic biomaterials. Finally, rapid intrusion of this extraspinal tumor into the dural sheath resulted in a confusing clinical picture and delayed the diagnosis and treatment of the tumor.

We report what we believe to be the first case of high-grade, radiation-induced, intratesticular leiomyosarcoma in a 30-year-old man who had had testicular relapse of acute lymphoblastic leukemia at age 12 years that was treated with standard testicular field radiation (2400 cGy) and chemotherapy. Radiation-induced tumors of this type are rare, have a median latency of 10 years, and are usually dose dependent (around 5000 cGy). Testicular leiomyosarcoma, especially high grade, remains to be fully characterized. After radical orchiectomy, patients should be followed up with serial germ cell tumor markers and imaging to monitor for metastatic spread. The use of retroperitoneal lymph node dissection and chemotherapy remains controversial but is probably not indicated.

We describe the clinical, radiologic, surgical, and pathologic findings of a 29-year-old Peruvian human immunodeficiency virus-infected man with a primary parasellar meningeal leiomyosarcoma involving the left lesser esphenoidal wing and the cavernous sinus. Over a period of 13 months, he developed headache, vomiting, insomnia, and diplopia. Magnetic resonance imaging revealed a left parasellar extra-axial mass that was isointense in T1, hypointense in T2, and gadolinium-enhanced. The patient underwent subtotal resection of the tumor. The neoplasm was composed of spindle cells with smooth-muscle features. It showed moderate atypia, inconspicuous nucleoli, and scanty mitosis. No tumor necrosis was detected. The immunohistochemistry revealed strong positivity for vimentin, desmin, and smooth-muscle alpha-actin. A low-grade leiomyosarcoma was diagnosed. The in situ hybridization showed positive nuclear reactivity for Epstein-Barr virus-encoded RNA. The immunohistochemistry was negative for Epstein-Barr virus latent membrane protein 1. The main differential diagnosis of primary meningeal smooth-muscle tumors includes meningioma and peripheral nerve sheath tumors. Epstein-Barr virus has been demonstrated in most smooth-muscle tumors associated with acquired immune deficiency syndrome (AIDS). Primary meningeal smooth-muscle tumors, exceedingly rare neoplasms, remarkably affect young adults with AIDS. Comparatively, most AIDS-related visceral (nonmeningeal) smooth-muscle tumors have been reported in children. The permissiveness and tumorigenesis associated with Epstein-Barr virus may depend on the age of human immunodeficiency virus infection.

Introduction: Primary malignant lesions of the vagina represent less than 2% of all gynecologic malignancies. Primary vaginal sarcomas account for about 2% of all malignant vaginal lesions, with leiomyosarcoma being the most common vaginal sarcoma found in adult women., Case: We report a case of primary vaginal leiomyosarcoma occurring in the field of a prior tension-free vaginal tape (TVT) procedure using a Bard Duraderm allograft., Conclusion: This report represents the first report, to our knowledge, of a vaginal sarcoma arising in the field of a Bard Duraderm TVT allograft. Although the product is no longer available for this use, the ongoing studies of the TVT procedure and outcomes should include this potential complication in their review.

The frequency of membranous lupus nephritis recurrence (World Health Organization (WHO) class V) in the allograft after renal transplantation is unknown, but it appears uncommon (only two reported cases in the literature). Despite the increased incidence of sarcomas in organ transplant recipients (compared to the general population), non-Kaposi's sarcoma is an uncommon malignancy, and primary tumor involvement of a renal allograft is a rare occurrence. Our patient is a 28 year old female with end-stage renal disease (ESRD) secondary to membranous lupus nephritis who received a living related transplant from her mother. At 26 months post-transplant, she presented with proteinuria and a rise in creatinine (Cr). Allograft biopsy was consistent with recurrent membranous nephropathy. Five weeks later, she was found to have a high-grade leiomyosarcoma originating within the allograft. We reviewed the literature on recurrent post-transplant membranous nephropathy and the possible role of the Epstein-Barr virus (EBV) infection in smooth muscle tumors occurring in organ transplant recipients. We also considered the association of membranous nephropathy and malignancy.

Objective: To report our incidence of soft tissue tumors at this site and to discuss various prognostic factors., Methods: All patients with a diagnosis of vulvar soft tissue neoplasms were studied from a prospective database at the Royal Marsden Hospital between January 1985 and July 2001., Results: Seventeen vulvar soft tissue neoplasms (11 malignant and six benign) were treated during this period. Leiomyosarcoma (n = 5) and aggressive angiomyxoma (n = 4) were the most frequent histologic types. According to the grade, there were four G3, three G2, three G1 and could not be assessed in one patient. Local recurrence occurred in six patients with sarcoma (three with high grade and one each with intermediate, low, and undetermined grade). In this group, five patients had negative microscopic margins and one patient had positive microscopic margins on excision. All three women with low-grade sarcomas are alive without evidence of disease. Three patients with aggressive angiomyxoma also had a local recurrence., Conclusion: Surgical excision is the primary treatment where possible. The grade of the tumor is an important predictor for local recurrence and outcome. Aggressive angiomyxoma is a local problem. Leiomyosarcoma and aggressive angiomyxoma are the most frequent histologic types.

Central nervous system leiomyosarcomas are extremely rare, however, they became more frequent among immunodeficient patients, either in a patients infected with human immunodeficiency virus (HIV), or after organ transplantation. The data of the literature indicate that the infection by Epstein-Barr virus (EBV) plays a causal role in the development of these tumours but its precise role in the oncogenesis remains unresolved. We report a new case of EBV associated leiomyosarcoma of the left cavernous sinus occurring after renal transplantation. The epidemiological, clinical, pathological and therapeutic characteristics of these tumours are discussed.

Context: Although cancers occur with increased frequency in children with human immunodeficiency virus (HIV) infection, the specific clinical, immunological, and viral risk factors for malignancy have not been identified., Objective: To identify risk factors for malignancy among HIV-infected children., Design, Setting, and Patients: A multicenter case-control study of children with HIV at 26 institutions participating in the Pediatric Oncology Group. Forty-three case patients with a new malignancy and 74 control patients without a malignancy were matched based on the duration of their infection. Patients were enrolled between January 1992 and July 1998., Main Outcome Measures: Clinical and laboratory factors assessed as putative risk factors included demographic characteristics, HIV characteristics, prior antiretroviral treatment, and CD4 cell count. Coviral infections with Epstein-Barr virus (EBV), cytomegalovirus, and human herpesvirus 6 were assessed by semiquantitative polymerase chain reaction assays and serological testing., Results: Case malignancy diagnoses included 28 non-Hodgkin lymphoma, 4 B-cell acute lymphoblastic leukemia, 1 Hodgkin disease, 8 leiomyosarcoma, 1 hepatoblastoma, and 1 schwannoma. Epstein-Barr virus viral load of more than 50 viral genome copies per 105 peripheral blood mononuclear cells was strongly associated with cancer risk but only for children with CD4 cell counts of at least 200/ microL (odds ratio [OR], 11.33; 95% confidence interval [CI], 2.09-65.66, P<.001). High EBV viral load was not associated with cancer for children with CD4 cell counts of less than 200/ microL (OR, 1.12; 95% CI, 0.13-9.62; P =.99). Zidovudine antiretroviral therapy did not confer a significant protective effect for either the high (OR, 0.81; 95% CI, 0.22-3.09; P =.77) or the low CD4 cell count groups (OR, 0.27; 95% CI, 0.04-1.46; P =.16). The route of HIV infection was not associated with increased cancer risk., Conclusions: Route of infection, demographic characteristics, and zidovudine use were not associated with the development of malignancy in HIV-infected children. High viral burden with EBV was associated with the development of malignancy in HIV-infected children although the effect was modified by CD4 cell count. The pathogenesis of HIV-related pediatric malignancies remains unclear and other contributing risk factors can be elucidated only through further study.

The epidemiology of gastric cancer is remarkable for both its dramatic decline in incidence over the past century and its continuing presence as the second leading cause of cancer deaths worldwide despite this decline. Factors including increased consumption of fruits and vegetables, and decreased intake of salty foods have largely been credited for the decline. Epidemiologic studies continue to provide data on other gastric cancer risk factors, including associations with Helicobacter pylori infection, as well as dietary factors, tobacco, and alcohol intake. In response to the opposing trends of decreasing distal gastric cancer and increasing gastric cardia adenocarcinoma, studies are beginning to identify gastric cancer risk factors separately by tumor subsite. Future epidemiologic studies that include information on site of origin as well as molecular markers promise to yield more homogeneous classification of case groups, which will enhance identification of underlying disease processes., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Purpose of Investigation: The objective of this retrospective multicenter study was to assess the prognostic relevance of histologic type in uterine sarcomas., Methods: The hospital reports of 249 patients with uterine sarcomas were reviewed. Surgery was the initial therapy for all patients. Histologic type was leiomyosarcoma in 95 cases, low-grade endometrial stromal sarcoma (ESS) in 19, high-grade ESS in 34, and carcinosarcoma in 101. Postoperative treatment was given without well-defined protocols. Median follow-up of survivors was 97 months., Results: In the whole series 2-year, 5-year, and 10-year survival rates were 53.5%, 41.6%, and 35.8%, respectively, and median survival was 31 months. At univariate analysis survival was significantly related to stage (p = 0.0001), mitotic count (p = 0.0001), and histologic type (low-grade ESS vs leiomyosarcoma vs carcinosarcoma vs high-grade ESS, median: not reached vs 27 months vs 21 months vs 16.5 months, p = 0.0011), but not to postoperative therapy and patient age. The Cox model revealed that tumor stage, mitotic count and histologic type were independent prognostic variables for survival. In detail, the risk of death was significantly lower for low-grade ESS (risk ratio [RR] = 0.257; 95% confidence interval [CI] = 0.071-0.931) and carcinosarcoma (RR = 0.509; 955 CI = 0.324-0.799) when compared to leiomyosarcoma. Conversely, no significant difference in survival was found between leiomyosarcoma and high-grade ESS., Conclusions: Histologic type is an independent prognostic variable for survival in uterine sarcomas. Low-grade ESS has the best clinical outcome, whereas leiomyosarcoma has the poorest one. It is noteworthy that, when adjusting for stage and mitotic count, leiomyosarcoma has a significantly worse prognosis than carcinosarcoma.

We report an extremely rare case of leiomyosarcoma arising from a remnant esophagus. A 52-year-old Japanese man was referred to our hospital for treatment of a tumor arising from the remnant esophagus. Four years earlier, he underwent a subtotal esophagectomy for esophageal squamous cell carcinoma (well differentiated squamous cell carcinoma, T1N0M0 Stage I) located in the lower esophagus. After preoperative studies, partial esophagectomy with laryngeal preservation and reconstruction using a free graft from the jejunum were performed. Histopathological and immunohistochemical examination revealed leiomyosarcoma without metastasis. Immunohistochemical examination showed that most tumor cells were positive for smooth muscle actin and vimentin, but were negative for cytokeratin and S100. The deeply biopsied specimens are helpful for preoperative histological diagnosis. Mitotic activity has been considered an important criterion of malignancy. However, some cases with minimal mitosis in the tumor grow rapidly and were associated with poor prognosis. Therefore, we advocate that the clinical behavior is the only true indication of malignancy. We also provide a review of 64 cases of esophageal leiomyosarcoma reported in the Japanese literature with available data between 1969 and 1999, including the present case, and discuss their clinicopathological features. Asynchronous occurrence of leiomyosarcoma and squamous cell carcinoma in the esophagus is most unusual and has never been reported. Patients with infiltrating type leiomyosarcoma measuring more than 5 cm in diameter tend to have a poor prognosis. Chemotherapy did not exhibit any survival benefits. In the present patient, no recurrence has been noted for 23 months after surgery.

Multiple primary tumors occur more commonly in the region of the head and neck than elsewhere in the body. The chance of this is particularly high in patients treated for retinoblastoma, in part because of a genetic predisposition, and in part because of the possibility of irradiation treatment. However, triple tumors occur in only 0.5% of multiple tumors. A rare case of a triple (metachronous) tumor is reported: 12 years after the treatment of bilateral retinoblastoma (enucleation and irradiation), secondary leiomyosarcoma developed in the maxillofacial region, followed 5 years later by choriocarcinoma. Surgery was performed on all three types of tumor. As a result, the female patient (currently 21 years old) is now free of complaints and has married. It is extremely rare for either leiomyosarcoma or choriocarcinoma (CHC) to appear in the maxillofacial region. The long-term, systematic control of such patients is absolutely necessary, for the multiple tumors tend to develop only after a long latency period of 10 to 20 years.

We report a 5.5-year-old boy who underwent autologous peripheral blood stem cell transplantation for high-risk acute lymphoblastic leukemia and who had two abdominal masses develop 6 months later. Macroscopically complete resection of the abdominal tumors was performed and revealed a well-differentiated leiomyosarcoma. Smooth muscle tumors, benign or malignant, are increasingly recognized in children with various immunodeficiencies; the association with acute lymphoblastic leukemia is rarely described.

We report herein a case of leiomyosarcoma of Meckel's diverticulum which presented as a rare manifestation of perforation. A previously healthy 63-year-old man was referred to the Tetsujinkai Eniwa hospital following the sudden development of acute abdominal pain. Abdominal computed tomography revealed a solid mass in the pelvic cavity, and an emergency operation was performed under a provisional diagnosis of peritonitis associated with a pelvic tumor. A perforated tumoral mass was found in Meckel's diverticulum. Segmental resection of the ileum, including the tumor-bearing diverticulum, was performed along with regional lymph node dissection. Histologic examination revealed the lesion to be leiomyosarcoma.

Leiomyosarcoma of the scrotum is a rare tumor. Up to 1999, only 29 cases have been reported in the literature worldwide. A 27-year-old man presented with a mass on the left side of the scrotum which had been painless and had gradually enlarged over the previous 10 years. During the following 3 months, however, it became painful and he was then referred to our hospital. Physical examination revealed a solid mass on the left side of the scrotum, measuring 7 cm in diameter, which was not adhering to the testis or to the vas deferens. The tumor was surgically resected. The histological examination confirmed the diagnosis of well differentiated leiomyosarcoma. Adjuvant therapy was considered unnecessary. The follow-up at 41 months revealed no local recurrence or distant metastasis.

We describe a patient who presented with autoimmune hemolytic anemia and small bowel obstruction secondary to a malignant stromal tumor (leiomyosarcoma) of the jejunum, 25 years postchemotherapy and radiation treatment for stage IIA Hodgkin's disease. The patient was treated with corticosteroid therapy and surgical resection of the jejunal tumor. We conclude that autoimmune hemolytic anemia may be an unusual presentation for postirradiation sarcoma.

Epstein-Barr virus (EBV)-associated smooth muscle tumors following solid organ transplantation are extremely rare, with only 12 cases reported in the literature thus far. The exact pathogenetic role of EBV infection in the oncogenesis of these soft tissue tumors in immunodeficient patients and the biologic behavior of such tumors is still unclear. We report a 26-year-old man in whom multiple smooth muscle tumors developed 36 to 51 months after heart transplantation. All tumors, two synchronous liver nodules, two subsequently occurring paravertebral tumors, and a single tumor in a vein at the left ankle were surgically resected. The tumor tissue was processed for routine histology and immunohistochemical (IHC) stains. Additionally, competitive polymerase-chain-reaction (PCR), reverse-transcriptase PCR (RT-PCR), as well as in situ hybridization (ISH) were used for EBV particle quantification and gene transcription analysis. The histologic features and immunohistochemical profiles were consistent with leiomyosarcoma in all tumor nodules. EBV infection was detected in >95% of tumor cell nuclei by EBER 1/2 ISH. Competitive PCR revealed 3105 EBV particles per milligram of tumor tissue. The EBV gene expression pattern analyzed by RT-PCR and IHC corresponded to the latency type III with specific expression of EBNA1, EBNA2, LMP1, and LMP2A genes. Under continuous antiviral therapy (famcyclovir) the patient currently shows no evidence of disease. Our data indicate that EBV infection plays a causal role in the development of smooth muscle tumors following organ transplantation. A latency type III, identical to EBV-associated posttransplant lymphoproliferative disorders, was identified and suggests a common pathogenetic mechanism in the development of these histogenetically distinct neoplasms. The fact that the patient currently shows no evidence of disease may be the result of the continuous administration of antiviral therapy because the soft tissue recurrences of the leiomyosarcoma occurred while the patient was not receiving antiviral prophylaxis.

Second solid tumors are well known late complications after bone marrow transplantation. Treatment strategies are ill defined. We retrospectively evaluated treatment and outcome in a single institution. From August 1974 to July 1996, six solid tumors were observed in five of 387 patients 2 to 13 years after BMT, corresponding to a probability of developing a second solid tumor of 9% (1-17%, 95 CI) at 15 years: these comprised endometrial carcinoma, carcinoma of the thyroid gland, cervical carcinoma, sarcoma of the small intestine, osteosarcoma of the tibia and ovarian carcinoma. All five patients were treated as intensively as they would be without a history of BMT. At last follow-up four of the five patients were alive and without signs of tumor. We postulate that second solid tumors after BMT should be treated as de novo tumors. Early detection based on consequent clinical follow-up of the transplant patients might explain the relatively good outcome.

The authors report a case of mesenchymal tumor (leiomyosarcoma) arising in a Meckel's diverticulum, with an history of digestive bleeding. Different kinds of Meckel's diverticulum tumors, their symptomatology and diagnostic difficulties are then discussed.

In summary, cutaneous malignancies with an epithelioid appearance form a diverse group of neoplasms that may be difficult to diagnose by utilizing routine microscopy alone. Cutaneous malignancies, including malignant melanoma and metastatic carcinoma, certain benign neoplasms such as mixed tumor of the skin and angiolymphoid hyperplasia with eosinophils (epithelioid hemangioma), and infectious conditions such as bacillary (epithelioid) angiomatosis can be considered in this differential. However, through recognition of the characteristic histologic, immunocytochemical, and ultrastructural findings outlined above, definitive diagnosis of these challenging neoplasms is usually possible.

We report a case of primary low-grade leiomyosarcoma of the mandible in an otherwise healthy young woman. The neoplasm presented as a painful, pericoronal gingival swelling that mimicked an acute periodontal infection. It was managed accordingly, with curettage, debridement, and antibiotics. When the lesion failed to respond to this treatment approach, a biopsy was performed. Microscopy revealed a malignant mesenchymal neoplasm which, on immunohistochemistry analysis, demonstrated reactivity for smooth muscle actin (SMA) and vimentin. This established the diagnosis of leiomyosarcoma; subsequently, an en bloc resection of mandibular bone and overlying soft tissue was performed. Close follow-up for over 10 years has revealed no evidence of recurrent or metastatic disease. Since the patient was taking oral contraceptives prior to the onset of the lesion, a possible link between estrogen and smooth muscle tumors is considered.

Leiomyosarcoma has recently been identified as an AIDS-related tumor, usually occurring in children in the chest or abdomen. We describe a case of intracranial leiomyosarcoma in an adult with AIDS. An enhancing mass adjacent to the right occipital cortical surface was diagnosed during a work-up for left arm weakness. Imaging characteristics were suggestive of a malignant neoplasm versus meningioma. The patient had a craniotomy for total excision of the lesion. Pathology showed a leiomyosarcoma. Metastatic work-up was negative. The patient refused radiation therapy, but is well without evidence of recurrence at 8 months follow-up. The differential diagnosis for intracranial lesions in AIDS should be expanded to include leiomyosarcoma. Surgical resection of the lesion is recommended.

A 31-year-old man underwent total resection for a fibrillary astrocytoma in the right frontal lobe followed by 6MV x-ray radiotherapy. The portal field size was a square of 8 cm x 7 cm, and the total dose of irradiation was 50Gy, with single fractions of 2Gy. For the next 6.5 years there was no recurrence of the astrocytoma. At 38 years of age, the patient noticed a subcutaneous mass in the scar of the previous operation and developed generalized convulsive seizures. MRI revealed a dural tumor within the previous radiation field, and the tumor was partially removed. Histologically, it was diagnosed as a leiomyosarcoma. This dural sarcoma satisfies the widely used criteria for definition of radiation-induced malignancies first described by Cahan et al. Both the clinical features and the possible histogenesis of this secondary tumor are briefly discussed.

Materials and Methods: The research has been carried out with the cooperation of Department of Otorhinolaryngology of the "Ospedale S.S. Trinita" in Cagliari, on 294 patients suffering from oral malignant tumours between 1984 and 1994. This kind of disease was studied in relation to age, sex, histological type, and afterwards the examination results were compared to other researchers' studies. All the 294 patients were recorded in the operating theatre's registers. Oral malignant tumours amount to about 45% of the neoplastic systemic pathology. Use and misuse of alcohol, smoking, repeated blows resulting in pre-cancerous lesions including leukoplakia, might be considered the main causes of the origin of oral tumours., Results: Male patients (237) amount to 80.7% of the examined sample, while female ones (57) reach only 19.3%. The part most frequently involved is the tongue (97 cases, 33%), then the area of the lower lip (69 cases, 23.5%), and finally the lower part of the oral space (47 cases, 15.58%)., Conclusions: From the histological point of view, the squamous carcinoma is found more often in the tongue, in the lower oral space and in the lips, while the basal carcinoma in the lower lip rather then in the upper one. A remarkable increase in the squamous carcinoma and a grow in the areas of the tongue has been observed.

We report a case of radiation-induced retroperitoneal leiomyosarcoma which developed 37 years after the patient received radiation therapy for testicular seminoma. The sarcoma originated within the para-aortic field, extensively involving neighboring organs, soft tissue and muscle tissues, and could be only partially resected. The absolute number of these secondary sarcomas is low, but the risk of developing such neoplasms calls for awareness in the long-term follow-up of cured seminoma patients.

The pediatric AIDS epidemic began in the U.S.A. between 1983 and 1985. Hemophilia patients were among the first victims of this disease with the majority of these patients infected prior to 1984. At the South Texas Hemophilia Center 69 of 108 patients less than 21 years of age demonstrated serologic evidence of infection. Of these patients, 6 subsequently developed malignancies between 1987 and 1994. Between 1992 and 1996 data was subsequently accumulated on the development of malignancy in HIV positive patients through the Pediatric Oncology Group, which to date has enrolled 24 HIV positive children with malignancy. In these studies the majority of patients had B cell, non-Hodgkin's lymphomas, however approximately 20% of the patients were identified with leiomyosarcomas. Histologic studies of tumors of 6 children with AIDS and leiomyosarcomas or leiomyoma identified the EBV receptor or CD 21 in the tumor using immunoperoxidase techniques, whereas similar staining was not seen in smooth muscle tumors from HIV negative children. In situ hybridization techniques identified EBV-EBER probe in the tumors from HIV positive patients. In 2 patients with adequate tumor tissue EBV genome was present in high concentration using PCR techniques and Southern blot studies showed a monoclonal and biclonal proliferation. Other laboratories have reported similar EBV findings in lymphomas from AIDS patients. Thus EBV appears to be an important cofactor in development of malignancy in pediatric AIDS patients.

The magnitude for and risk factors of the two most important AIDS neoplasm, Kaposi's sarcoma and non-Hodgkin's lymphoma, are reviewed in detail. The association between AIDS and other cancers is mostly speculative because surveillance biases tend to favor detecting associations that may be spurious. The overall relative risk of other cancers appears, however, to be only twofold above that in the general population, with associations being most convincing for anal (but not cervical) cancer and leiomyosarcoma and possible also for Hodgkin's disease, testicular cancer, and conjunctival cancers.

Malignancies in children with HIV infection have not been as frequent as expected, but they still constitute a fertile area for clinical and basic research. Non-Hodgkin's lymphomas are the most frequent malignancies of children with AIDS and are curable diseases with standard chemotherapy. Leiomyomas and leiomyosarcomas have become the second leading cancer of children with HIV infection and are clearly associated with EBV infection. Treatment for these lesions has not been as successful as that for lymphomas. Other infrequent atypical lymphoproliferative lesions of these patients can often be categorized in the MALT group. Some of these are low-grade lymphomas, whereas others can progress to high grade. The diagnosis of Kaposi's sarcoma in children with AIDS should be carefully reviewed by pathologists experienced with these cases. The diagnosis of KS in children must be made with special care, because some other lesions of HIV-infected children (such as prominent vascularity in lymph nodes) can be confused with KS. Other tumors of these patients are rare and probably are no more frequent than would be expected in the normal population. Because malignancies in children with AIDS are rare, it is important that each one be studied completely with regard to type and incidence, risk factors, and biologic features. To this end, the Pediatric Oncology Group (POG) has established a national registry and treatment protocols. Patient information as well as fresh, frozen, and fixed specimen studies are coordinated through the POG Statistical Office in Gainesville, Florida (telephone, 904-392-5198; FAX, 904-392-8162). The collaborative efforts of all physicians treating children with AIDS and malignancies will be needed to advance our knowledge and efficacy in treating these diseases.

We report a rare primary adrenal leiomyosarcoma in a 30-year-old, human immunodeficiency virus--positive man. This is, we believe, the third documented case in the English literature of this tumor in this site, and the first to be reported in an adult male with acquired immunodeficiency syndrome. The smooth muscle origin of this tumor was apparent by routine microscopy and confirmed by positive immunostaining for smooth muscle actin. The patient is presently well and shows no evidence of recurrence 20 months after surgery. The present findings are discussed with reference to the reported rising incidence of smooth muscle tumors in human immunodeficiency virus--infected patients and the associated etiologic role of Epstein-Barr virus in the pathogenesis of these tumors.

A 77-year-old man was admitted to our hospital complaining of gross hematuria. Cystoscopy showed an approximately 4-cm non-papillary tumor in and out of the diverticulum of the left posterior wall. Total cystectomy was performed. Histopathological diagnosis was pleomorphic leiomyosarcoma. According to TNM classification of bladder cancer, the stage of this tumor was pT3bpN0M0. The patient had local recurrence two months after the operation, and died a month later. This is the second case of leiomyosarcoma of the diverticulum of urinary bladder reported in Japan.

Soft tissue sarcomas in the head and neck are rare. Aetiological factors relating to these tumours have not yet been identified. The association with von Recklinghausen's disease and with irradiation is however well recognized. In the literature it has been speculated that trauma may also play a role in the development of soft tissue sarcomas. In this article we present five patients with a history of surgical trauma at the site where a sarcoma later developed. Although we cannot prove a causal relationship, the relatively high incidence of possibly trauma-related soft tissue sarcomas in a series of 60 patients we have seen over a 30-year period, suggests that such a relationship could exist.

A 48-year-old female patient with von Recklinghausen's disease was admitted to Kaohsiung Medical College Hospital due to massive hematochezia. The emergent mesentery arteriography revealed multiple hypervascular small intestinal lesions but no contrast extravasation. The endoscopic examination revealed a blood-filled small intestine with normal colonic mucosa. Laparotomy was carried out and multiple extraluminal masses on antimesenteric side of the small intestine were found. Segmental resection of the small intestine resulted in resolution of her symptom. The largest mass was diagnosed as leiomyosarcoma, and the others were diagnosed as leiomyoma. Histologically, differential diagnosis between leiomyosarcoma and leiomyoma is not clear cut, because it is based on a number of differentiating features. Surgical resection is the treatment of choice for intestinal leiomyosarcoma. The long-term follow-up of this patient is mandatory.

A patient with a sarcoma of the left groin at a site where a prior seminoma had been irradiated is presented. Very few postirradiation sarcomas are described in patients treated with radiation therapy for testicular cancers, even if an increased risk of second malignancies has been reported. In contrast with the highly aggressive clinical course of postirradiation sarcomas, an early discovery of the lesion allowed a radical surgical approach with a presumable cure of the patient. A continuous and precise follow-up of patients irradiated for seminoma is suggested.

Two cases of smooth muscle tumor developing in immunocompromised children are described. Both children presented after transplant surgery, one with a hepatic leiomyoma and the other with a hepatic leiomyosarcoma.