Neglected orthopedic oncology ‑ Causes, epidemiology and challenges for management in developing countries.

BACKGROUND: Management of malignant bone and soft tissue tumors remains an overwhelming confront to orthopedic surgeons. The challenge is discriminating in developing countries due to inadequate diagnostic and therapeutic amenities and unawareness. A lot has been discussed about the neglected orthopedic trauma, but the published literature on the causes and management of neglected bone and soft tissue tumors is sparse. Hence, current study was undertaken to highlight the causes of neglect and therapeutic challenges for managing these neglected tumors in developing countries. AIMS AND OBJECTIVES: To determine the causes of neglect of malignant bone and soft tissue tumors, their epidemiology (including their relative frequencies, age, gender discrimination, anatomical sites of occurrence and histological characteristics) and difficult aspect of management due to neglect or delayed presentation. MATERIALS AND METHODS: This was an appraisal of the neglected malignant bone and soft tissue tumors presented to J. N. Medical College and Hospital from June 2008 to May 2013. Criteria for labeling the tumor as neglected malignant bone and soft tissue tumor was delayed presentation (>3 months), locally advanced disease, ulceration, sepsis, fungating mass or metastasis at the time of presentation. All the cases were reviewed and analyzed for age, gender, histological types, educational status and socioeconomic status of the family, any prior treatment by traditional bone setters or registered medical practitioner, cause of delay for seeking medical advice. We have also analyzed the treatment given at our institute and the outcome of the tumor. OBSERVATIONS AND RESULTS: Eighteen patients fulfilled the criteria for neglected malignant bone and soft tissue tumors, hence were included in study. Eight cases were of osteosarcoma, five cases were of Ewing’s sarcoma, three cases were of chondrosarcoma and 1 case each was of pleomorphic liposarcoma and primary lymphoma of bone. According to Enneking staging system 11 cases were of stage III (distant metastasis) and 7 were stage II‑B. Seven were females, and 11 were males. Age range was 5–68 years. 15 patients (83.3%) belonged to low socioeconomic status with 17 patients (94.4%) belonged to uneducated background. Cause of delay in seeking medical advice was neglect by the patient and family due to financial constraints, cultural and religious believes, lack of access to health care facilities, consultation with traditional bone setters and even misdiagnosis by qualified orthopedic surgeons. The tumors included were all unresectable and of huge sizes, hence were managed with amputation/dis‑articulation, chemotherapy or radiation. CONCLUSION: The current study tries to highlight the causes and quantity of neglect of malignant bone and soft tissue tumors prevalent in our country, which poses a therapeutic challenge for management and consequent mutilating surgeries with poor outcome resulting in loss of extremity and existence.

Liposarcoma intramuscular del muslo tratado con braquiterapia de alta tasa de dosis. Reporte de un caso / Intramuscular thigh liposarcoma treated with high dose rate brachytherapy. A case report

El liposarcoma es un tumor maligno de origen mesenquimal. Comparado con otros tipos de neoplasias malignas, los sarcomas de partes blandas son tumores relativamente raros. Representan el 1% de todas las neoplasias y supone del 9 al 18% del total de sarcomas de partes blandas. Es una neoplasia propia de pacientes adultos, con mayor incidencia entre los 40 y los 60 años, con ligera preferencia del sexo masculino. Hasta hace tres décadas, la tendencia ante la presencia de este tipo de lesiones en las extremidades era la cirugía radical. Presentamos el caso de paciente femenino de 37 años quien presenta una tumoración en el muslo izquierdo. La biopsia incisional reportó una neoplasia maligna poco diferenciada. Ante la sospecha clínica y radiológica de la presencia de un liposarcoma fue llevada a quirófano donde se realizó compartamentectomía medial del muslo izquierdo, con colocación intraoperatoria de 12 catéteres para braquiterapia y linfadenectomía inguinofemoral izquierda. La braquiterapia es una forma de radioterapia adyuvante interna y una alternativa útil y atractiva(AU)

Liposarcoma is a malignant tumor of mesenchymal origin. Compared with other types of malignancies, soft tissue sarcomas are relatively rare tumors. Represent 1% of all malignancies and is from 9 to 18% of soft tissue sarcomas. Its an adult patients tumor, with the highest incidence between 40 and 60 years and with a slight prevalence of males. Until three decades ago, the trend in the presence of these lesions in the extremities was radical surgery. We report the case of a 37 years old female patient who has a growth on the thigh. Incisional biopsy reported a poorly differentiated malignant neoplasm. Suspecting a liposarcoma was taken to the operating room where left medial thigh compartamentectomy was made and intraoperative placement of 12 brachytherapy catheters and left inguinofemoral lymphadenectomy. Brachytherapy is a form of internal radiation and an alternative, useful and attractive adjuvant therapy(AU)

Osteosarcoma condroblástico gigante del fémur: reporte de un caso / Giant chondroblastic osteosarcoma of the femur: a case report

El osteosarcoma es un tumor maligno compuesto de células fusiformes, que se caracteriza por la producción de tejido osteoide y hueso, su crecimiento es rápido con extensión locoregional y difusión metastásica a pulmón. Generalmente 20% de los pacientes presentan metástasis en el momento del diagnóstico. Se observa con frecuencia en los huesos largos de los adolescentes y adultos jóvenes con mayor afectación del sexo masculino. El tratamiento de los pacientes con osteosarcomas se basa en la resección amplia y completa o en una amputación del tumor primario, y en la administración de quimioterapia coadyuvante. Presentamos el caso de paciente masculino de 21 años, quien consultó por una tumoración en la rodilla izquierda de rápido crecimiento hasta alcanzar 80 cms de perímetro. La biopsia incisional reportó un condrosarcoma condroblástico. En vista de no tener respuesta al tratamiento coadyuvante, se decidió realizar una desarticulación coxofemoral. Cuando el osteosarcoma es resistente a la quimioterapia y su crecimiento continúa, se deben realizar procedimientos radicales para garantizar la sobrevida del paciente(AU)

Osteosarcoma is a malignant tumor composed of spindle cells, characterized by the production of osteoid tissue and bone is growing rapidly, with locoregional extension and metastatic spread to the lung. Generally 20% of patients have metastases at diagnosis. It most often occurs in the long bones of adolescents and young adults with greater male involvement. Treating osteosarcoma patients is based on wide and complete resection or amputation of the primary tumor and the administration of adjuvant chemotherapy. We present the case of male patient 21, who presented a tumor in his left knee rapidly growing up to 80 cm in circumference. Incisional biopsy chondroblastic reported chondrosarcoma. In view of having no adjuvant treatment response was decided to hip disarticulation. When osteosarcoma is resistant to chemotherapy and growth continues, radical procedures must be performed to ensure the survival of the patient(AU)