Pleomorphic undifferentiated soft tissue sarcoma in patient with long standing inflammatory bowel disease

Inflammatory bowel disease (IBD) has been associated with the development of both gastrointestinal and extraintestinal malignancy. The role of therapy in the development of malignancy in IBD has been controversial. We present the case of a 40-year-old female patient with long-standing mild IBD, who developed an undifferentiated pleomorphic sarcoma of the inguinal region and provide a brief review of the relevant literature. While our case likely represents a coincidence of two unrelated pathological entities, clinicians should keep in mind the possibility of soft tissue sarcomas in patients chronically treated with anti-inflammatory agents.

Osteosarcoma primario de mama en una paciente con antecedentes de tratamiento radioterápico loco-regional por carcinoma in situ mamario / Primary breast osteosarcoma in a patient with a history of locoregional radiotherapy treatment for breast carcinoma in situ

RESUMEN El cáncer de mama es el tumor más prevalente a nivel mundial entre las mujeres. A pesar de que la supervivencia global alcanza cifras cercanas al 90%, sigue suponiendo un importante problema sanitario y económico para la población. Los sarcomas primarios de mama suponen menos del 1% de estas neoplasias, pero sus tasas de recidiva y mortalidad son elevadas. Dado que no existen pruebas de imagen específicas para el diagnóstico, la confirmación de dicha entidad supone un reto a nivel histopatológico. El tratamiento del osteosarcoma de mama es principalmente quirúrgico, con la extirpación de la tumoración con márgenes adecuados, que será trascendental para el pronóstico de las pacientes. No se ha demostrado que sea necesaria una disección axilar, ni un tratamiento adyuvante estándar de quimioterapia ni radioterapia, salvo casos indicados. Presentamos el caso de una paciente postmenopaúsica que tras haber desarrollado un carcinoma ductal in situ de mama tratado de forma satisfactoria, desarrolla dos años posterior a la finalización del tratamiento radioterápico, y en la misma localización del tumor inicial, un osteosarcoma primario de mama de rápido crecimiento atribuible al efecto de la radioterapia administrada.

ABSTRACT Breast cancer is the most prevalent tumor worldwide among women. Despite the fact that overall survival reaches figures close to 90%, it continues to be an important health and economic problem for the population. Primary breast sarcomas account for less than 1% of these neoplasms but their rates of recurrence and mortality are high. Given that there are no specific imaging tests for diagnosis, confirmation of this entity is a challenge at the histopathological level. The treatment of the OSM is mainly surgical with the removal of the tumor with adequate margins, which will be transcendental for the prognosis of the patients. It has not been demonstrated that an axillary dissection is required, nor a standard adjuvant treatment of chemotherapy or radiotherapy, unless indicated. We present the case of a postmenopausal patient who, after having developed a ductal carcinoma in situ of the breast (DCIS) successfully treated, developed two years after the end of the radiotherapy treatment, and in the same location of the initial tumor, an osteosarcoma primary breast of rapid growth attributable to the effect of radiotherapy administered.

Sarcoma miofibroblástico posradioterapia / Radiation-induced myofibroblastic sarcoma

Paciente de 51 años, varón, fumador y alcohólico moderado, con síntomas de disfonía intermitente que debuta con una adenopatía cervical metastásica de carcinoma epidermoide, sin hallazgos del tumor primario. Es intervenido quirúrgicamente siéndole realizada una disección cervical unilateral y posteriormente es tratado con quimiorradioterapia concomitante. A los 4 años del final del tratamiento oncológico, sin recidiva local ni aparición del tumor primario, el paciente debuta con disnea de presentación subaguda. Tras la exploración física y pruebas diagnósticas, se constata una nueva tumoración en laringe que resulta ser un "sarcoma miofibroblástico de bajo grado". Según los criterios de Cahan, este tumor, de estirpe histológica poco frecuente en laringe, hace pensar que probablemente podría ser inducido por la radiación. Se discuten los criterios de causalidad entre radiación y oncogenesis y se resumen las características de los tumores radioinducidos.

We report a clinical case about a male patient, smoker and alcoholic, that debuts with a neck metastasis of an epidermoid carcinoma of unknown origin. He was submitted to a cervical dissection and treated with adjuvant combined chemo and radio therapy. Four years after finishing the oncologic treatment without tumor recurrence or appearence of the primary tumor, he begins with sub acute dyspnea. After physical examination and imaging, a new larynx tumor was found, "low grade myofibroblastic sarcoma". Under Cahan criteria, this tumoral kind, rare in larynx, was probably induced by the local radiotherapy. We discuss briefly the causality between radiation and oncogenesis and the characteristics of the radio induced tumors.

Sarcoma indiferenciado primário no sistema nervoso central / Primary undifferentiated sarcoma of the central nervous system

INTRODUÇÃO: O sarcoma de sistema nervoso central (SNC) é uma neoplasia rara, com incidência de 0,1% a 4,3% dos tumores intracranianos. São tumores agressivos com prognóstico reservado e a maioria é tratada com ressecção radical. RELATO: Homem, 29 anos, com episódios de crises convulsivas e diagnóstico de hemorragia intraparenquimatosa. Durante a cirurgia, foi identificada lesão bem delimitada. A histologia demonstrou neoplasia fusocelular com atipias e numerosas mitoses. Os únicos marcadores imuno-histoquímicos positivos foram vimentina e S-100. O diagnóstico foi de sarcoma indiferenciado de alto grau. CONCLUSÃO: No diagnóstico diferencial de sarcomas de SNC, devem-se excluir lesões metastáticas e gliossarcoma.

INTRODUCTION: The central nervous system (CNS) sarcoma is a rare neoplasm with an incidence of 0.1% to 4.3% in intracranial tumors. They are aggressive with poor prognosis, and mostly treated with radical resection. REPORT: 29 year-old male patient with episodes of seizures and diagnosed with intraparenchymal hemorrhage. During the surgery a well-defined lesion was identified. Histology showed a spindle cell neoplasm with atypia and numerous mitoses. The immunohistochemical markers were positive only for vimentin and S-100. The diagnosis was high-grade undifferentiated sarcoma. CONCLUSION: Metastatic lesions and gliosarcoma should be excluded in the differential diagnosis of CNS sarcomas.

Radiation-induced spindle cell sarcoma: A rare case report.

Ionizing radiation has been known to induce malignant transformation in human beings. Radiation-induced sarcomas are a late sequel of radiation therapy. Most sarcomas have been reported to occur after exposure to a radiation dose of 55 Gray (Gy) and above, with a dose ranging from 16 to 112 Gys. Spindle cell sarcomas, arising after radiotherapy given to treat the carcinoma of head and neck region is a very uncommon sequel. This is a rare case report of spindle cell sarcoma of left maxilla, in a 24-year-old male, occurring as a late complication of radiotherapy with Cobalt-60 given for the treatment of retinoblastoma of the left eye 21 years back.

A review of soft tissue sarcoma

Background : Soft tissue sarcomas (STS) are a heterogeneous group of rare tumours that arise predominantly from the embryonic mesoderm. They present most commonly as an asymptomatic mass originating in an extremity but can occur anywhere in the body; particularly the trunk; retroperitoneum; or the head and neck. They account for about 0.7of all adult malignancies. Method : A review of the literature of STS was undertaken with emphasis on current approach in management. Result : Despite recent advances in the knowledge of the molecular biology of STS; there is yet no identifiable aetiology in most cases. Tru-cut biopsy is a safe; accurate and economical procedure for diagnosing STS. Enough tissue is usually obtained for use in several diagnostic tests such as electron microscopy and cytogenetic analysis. With the advent of Computed Tomography (CT); Resonance Imaging (MRI) and Magnetic Resonance Angiography (MRA); tumours can easily be delineated from muscle groups; bone and neurovascular structures. Surgery remains the main potentially curative therapy for STS. In the last two decades; the role of adjuvant radiotherapy has revolutionized the treatment from a situation where amputation was the standard treatment for extremity STS to the present time where limb sparing surgery is appropriate in more than 90of patients. Postoperative adjuvant chemotherapy significantly improves the overall and disease free survival for patients with large size and high grade sarcomas. Conclusion : optimal results of treatment require multidisciplinary interaction between the referring practitioner; the treating surgeon; the pathologist; the radiotherapist and the chemotherapist.1

1470 cases of sarcoma referring to Imam Khomeini hospital during an 11-year period

Sarcoma is a malignant neoplasm arising in mesenchymal tissue. It comprises 1% of adult malignancies and 15% of childhood neoplasms. The present study was designed to review the clinical characteristics of 1470 sarcoma cases and related predictors of out come, relapse and survival. For this retrospective study, 1470 medical files of patients with sarcoma who were referred during 1991-2002 to Imam Khomeini Hospital were studied. Sarcoma comprised 11% of all cases referring to our hospital. The mean age of patients with sarcoma was 30 years and male to female ratio was 3/2. Bone to soft tissue sarcoma ratio was 3.1 in children and 1.3 in adults. Osteosarcoma, Ewing and rhabdomyosarcoma presented 83% of children tumors, however, in adults osteosarcoma, synovial sarcoma and malignant fibrous histocytoma [MFH] were the most common subtypes. The main prognostic factors for survival were tumor size, margin of surgery, neurovascular involvement in pathological report, initial metastasis and complete response to initial therapy. Adjuvant radiotherapy, small tumor size, curative surgery with chemotherapy and free surgical margins were significantly associated with reduced recurrence. Complete response to primary therapy is the main independent variable of overall survival of patients. Earlier diagnosis and experienced team including surgical, medical and radiotherapy oncologist is needed for better response and longer survival rate

Granulocytic sarcoma in patients with chronic myeloid leukemia

Granulocytic sarcoma is an unusual variant of myeloid malignancy most commonly encountered in the course of chronic or acute myeloid leukaemia. Of 60 patients of chronic myeloid leikaemia studied over 21 months, we encountered 6 [10%] cases of granulocytic sarcoma. Four of these had granulocytic sarcoma on their first presentation. All those who were receiving hydroxyurea did not fare well but one who was put on DAT [daunorubacin, cyftosine arabinocide, 6 thioguanine] regimen went into remission with complete disappearance of lesions

Sarcomas de partes moles no adulto / Soft tissue sarcomas in adult patients

Os autores apresentam uma revisäo sobre sarcomas de partes moles (SPM), mostrando que säo tumores raros, com etiologia e epidemiologia pouco conhecidas, apesar de recentes avanços no campo da genética e biologia molecular. O diagnóstico dos SPM é de fácil realizaçäo por sua localizaçäo preferencial em membros, tronco, cabeça e pescoço, porém, muitas vezes seu tratamento é retardado por falta de um encaminhamento correto e rápido do paciente. Quanto ao estadiamento, além do tamanho do tumor (T), comprometimento linfonodal (N) e metástases (M), o grau de diferenciaçäo (G) tem fundamental importância. Quanto ao tratamento, até a década de 70, a cirurgia era a única arma disponível, mas, nos últimos anos, como na oncologia de um modo geral, a abordagem multidisciplinar através da associaçäo da radioterapia e quimioterapia, os resultados de tratamento com controle local e preservaçäo do membro tornaram-se animadores. Porém, o controle sistêmico da doença ainda é um desafio para o oncologista

Melanoma maligno de partes blandas (MMPB) (sarcoma de células claras de tendones y aponeurosis) / Malignant melanoma of soft parts (clear-cell sarcoma of tendons and aponeuroses)

Se reporta 11 casos de melanoma maligno de partes blandas (sarcoma de células claras) diagnosticados entre 1980 y 1992 en el Instituto Nacional de Enfermedades Neoplásicas (INEN). La edad de los pacientes estuvo entre 15 y 52 años. Predominó el sexo masculino (7 de 11 casos). La localización en todos los casos fue en la extremidad inferior y de preferencia en el pie (6 de 11 casos). La forma de presentación tumor en todos los casos y dolor en el 55 por ciento. El diagnóstico se hizo en base a los criterios de Enzinger en preparaciones de hematoxilina-eosina y se hizo tinciones especiales para anticuerpo melanoma específico (HMB-45) y proteína S-100. Siete pacientes tuvieron algún tratamiento previo. En el INEN el tratamiento fue amputación en 7 casos, resección local amplia en 2 casos, quimioterapia en un caso y ningún tratamiento adicional en un caso. Nueve pacientes fallecieron entre 2 y 49 meses, uno está vivo a los 24 meses y uno se perdió de vista. Se concluye que melanoma maligno de partes blandas tiene una baja incidencia, se presenta como tumor de crecimiento lento de localización preferente en extremidades inferiores, un número importante de casos se presenta con metástasis (6/11) al momento del diagnóstico, el tratamiento debería ser cirugía radical, por la frecuencia de recurrencias y considerar la disección ganglionar regional por la alta posibilidad de metástasis ganglionar. La mortalidad es alta.

Histiocitoma fibroso maligno angiomatoide retroperitoneal: relato de caso / Retroperitoneal angiomated malignant fibrous histiocytoma: a case report

Apresenta-se o caso de uma paciente de sessenta anos com um volumoso histiocitoma fibroso maligno angiomatoide retroperitoneal. A presenca de massa abdominal palpavel era a unica queixa da paciente, que foi submetida a tratamento cirurgico. Discutem-se as caracteristicas histologicas, clinicas e biologicas, bem como o manejo e o prognostico deste tipo de neoplasia

Sarcoma de tejidos blandos inducido por radiación: presentación de un caso y revisión de la literatura / Sarcoma of muscles softs induced by radiation: report of a case and review of the literature

Se presenta la historia clínica de una paciente de 57 años de edad en quien se diagnosticó cáncer de mama a la edad de 40 años y recibió terapia de radiación después de la cirugía. Como presentó recurrencias después del tratamiento se le prescribió terapia citotóxica sistémica. Dieciocho años después del diagnóstico de tumor en la mama presentó otro tipo de tumor primario, de diferente tipo histológico, el cual tuvo un curso extremadamente agresivo y fatal