Complex presentation of anterior chest wall chondrosarcoma

Chondrosarcoma is the third most common primary bone malignancy, but its thoracic presentation is unusual compared to the pelvis and extremities. Chest wall chondrosarcomas are difficult to be surgically resected due to their proximity to neurovascular structures. We report the case of a 48-year-old man presenting with a history of chest bulging. Computed tomography showed a lesion of approximately 12 cm in the sagittal axis adjacent to the upper lobe of the left lung, compressing the upper lobar bronchus and causing parenchymal atelectasis. Biopsy revealed chondrosarcoma. The lesion extrinsically compressed the left pectoralis major muscle and invaded the left pectoralis minor muscle. After complete surgical resection, the patient was discharged on postoperative day 20. (AU)

Condrosarcoma costal con diseminación intraperitoneal / Costal Chondrosarcoma with Intraperitoneal Dissemination

RESUMEN El condrosarcoma es definido como un tumor maligno con diferenciación de cartílago hialino puro que puede presentar cambios mixoides, calcificación y osificación. El objetivo es mostrar el resultado del tratamiento de una diseminación peritoneal a partir de un condrosarcoma costal. Se realizó una revisión de la literatura, las indicaciones y técnicas en el tratamiento de la diseminación peritoneal del condrosarcoma y se presentan los resultados en una paciente diagnosticada e intervenida en el Hospital Clínico Quirúrgico "Hermanos Ameijeiras", entre enero de 2014 y diciembre de 2017. Paciente femenina de 46 años, que 7 años antes presentó un aumento de volumen en región costal baja izquierda y fue intervenida quirúrgicamente. Con el diagnóstico de condrosarcoma, se realizó una resección costal en la primera ocasión y luego, en dos oportunidades más por recidiva tumoral, en la última intervención se coloca una prótesis de polipropileno. Dos años después de la última cirugía, acude de nuevo con un aumento de volumen en la parte baja (región tóraco-abdominal, línea axilar), salvo este síntoma, exhibía un estado general excelente. La diseminación peritoneal del condrosarcoma es excepcional, muy poco reportado a nivel mundial y con pocas experiencias en su tratamiento. Se realizó técnica de resección multivisceral y peritonectomía con quimioterapia adyuvante posoperatoria. No hubo complicaciones relacionadas con el proceder y se realizó una segunda intervención extensa por recidiva a los 2 años(AU)

ABSTRACT Chondrosarcoma is defined as a malignant tumor with pure hyaline cartilage differentiation and that may be accompanied with myxoid changes, calcification, and ossification. The objective is to show the treatment outcome for peritoneal dissemination from a rib chondrosarcoma. A review of the literature was carried out, as well as the indications and techniques corresponding to the treatment of chondrosarcoma peritoneal dissemination. The outcomes are presented in a patient diagnosed and operated on at Hermanos Ameijeiras Clinical-Surgical Hospital, between January 2014 and December 2017. Female patient, 46 years old, who, seven years earlier, had presented increased volume in the left lower rib region and undergone surgery. With the diagnosis of chondrosarcoma, a rib resection was performed the first time, and then, on two more occasions due to tumor recurrence, a polypropylene prosthesis was placed in the last intervention. Two years after the last surgery, she returned with increased volume in the lower part (thoracoabdominal region, axillary line), except for the following symptom: she exhibited an excellent general condition. Theperitoneal dissemination of chondrosarcoma is exceptional, very little reported worldwide, and with little treatment experience. A multivisceral resection and peritonectomy technique was performed with postoperative adjuvant chemotherapy. There were no complications related to the procedure and a second extensive intervention was performed after two-year relapse(AU)

Condrosarcoma de la laringe: reporte de un caso y revisión de la literatura / Chondrosarcoma of the larynx: case report and literature review

El cáncer de laringe es un tumor relativamente raro en Ecuador (0,5/100000 varones). Las lesiones neoplásicas de laringe son usualmente epiteliales y el tipo histológico más frecuente es el carcinoma escamocelular. El tumor mesenquimal más común es el condrosarcoma. En los 31 años de existencia del Registro Nacional de Tumores de Ecuador éste es el primer caso de condrosarcoma de laringe registrado. Por este motivo, hemos decidido reportar el manejo diagnóstico y terapéutico de este caso.

Laryngeal cancer is a relatively uncommon tumor en Ecuador (0,5/100000 males). These neoplastic lesions usually epithelial and the most frequent histological type is squamous cell carcinoma. The most common mesenchymal tumor is chondrosarcoma. No case of this type of laryngeal tumor has been registered since the foundation of the National Cancer Registry of Ecuador thirty-one years ago. For this reason, we have decided to report the diagnostic and therapeutic management of his case.

Condrosarcoma tóraco-abdominal / Thoraco-abdominal chondrosarcoma

Chondrosarcomas represent the third primary malignant bone tumor. Costal location occurs in 12 percent, being the most common tumor of the ribs. It affects most frequently in the second and fifth decades of life. This tumor is histologically classified into grades 1, 2 and 3 in descending order of differentiation. For grade 2, with oncologic resection, there is a 10-year survival of 64 percent, and a 10 percent risk for metastasis. Radiotherapy has a limited therapeutic role and chemotherapy has not shown benefits. We report a case of a 45 years old woman, who presented with a symptomatic growing mass in the right rib cage, of three months of evolution, whose evaluation by magnetic resonance imaging and computed tomography shows a sarcomatous tumor with involvement of ribs, muscles, diaphragm, pleura, peritoneum and liver. A small amount of intraperitoneal free fluid was observed. A block resection was made, resulting a 16.8 cm long at the widest point chondrosarcoma, grade 2, with involvement of all layers of the wall, tumor microfoci in the liver capsule and malignant tumor cells in peritoneal fluid.

Los condrosarcomas representan la tercera neoplasia ósea maligna primaria. La ubicación costal representa el 12 por ciento, siendo el tumor más frecuente de las costillas. Se presenta con mayor frecuencia en la segunda y quinta décadas de la vida. Este tumor se clasifica histológicamente en grados 1, 2 y 3 en orden decreciente de diferenciación. Para el grado 2, resecado oncológicamente, se observa una sobrevida a 10 años del 64 por ciento; existiendo un 10 por ciento de riesgo de metástasis. La radioterapia tiene un escaso rol terapéutico y la quimioterapia no ha demostrado beneficios. Presentamos el caso de una mujer de 45 años que consultó por un aumento de volumen sintomático de la parrilla costal derecha, de tres meses de evolución, cuya evaluación a través de tomografía axial y resonancia magnética permitió delinear un tumor sarcomatoso de la pared con afectación de costillas, músculos, diafragma, pleura, peritoneo e hígado. Además se observaba escasa cantidad de líquido libre intraperitoneal. Fue resecado en block un condrosarcoma de 16,8 cm de eje mayor, grado 2, con compromiso de todos los planos de la pared, microfocos tumorales en cápsula hepática y células neoplásicas malignas en líquido peritoneal.

Tratamiento, complicaciones y sobrevida del condrosarcoma del húmero proximal / Treatment, complications and survival of the chondrosarcoma of the proximal humerus

Introducción: El condrosarcoma constituye el segundo tumor óseo en frecuencia. La ubicación en el húmero es menos frecuente que en el fémur y la pelvis, por lo que hay escasa bibliografía de los resultados obtenidos en el tratamiento de este tumor. Materiales y métodos: Se analizaron retrospectivamente 28 pacientes (10 varones y 18 mujeres) con diagnóstico de condrosarcoma localizado en el húmero proximal tratados entre 1988 y 2010. El seguimiento promedio fue de 6 años (rango 6 a 200 meses). La edad promedio de los pacientes fue de 48 años (rango 17 a 75 años). En esta serie hubo 13 condrosarcomas de grado I, 11 de grado II, 2 de grado III y 2 condrosarcomas desdiferenciados. Dos pacientes fueron desarticulados y en 26 pacientes se conservó el miembro. De este último grupo, en 5 se realizó curetaje con relleno y en los 21 restantes, resección del tumor con reconstrucción (10 aloprótesis, 5 aloinjertos osteoarticulares, 4 aloinjertos intercalares y 2 endoprótesis). Resultados: Hubo 2 recidivas locales y 2 pacientes de la serie murieron por su enfermedad (los dos condrosarcomas desdiferenciados). No hubo complicaciones en el grupo tratado de manera intralesional y fueron 8 en los tratados de forma extralesional. Se conservó el miembro en el 100 por ciento de los condrosarcomas de bajo grado y en el 50 por ciento de los de alto grado. La sobrevida fue del 100 por ciento a los 5 años en los casos de condrosarcoma de bajo grado y del 50 por ciento en los de alto grado. Conclusiones: Se obtuvo una alta tasa de conservación del miembro en el tratamiento del condrosarcoma del húmero proximal sin afectar el índice de sobrevida de los pacientes en esta serie.

Chondrosarcoma of the bone with nodal metastasis: The first case report with review of literature.

Primary bone sarcomas are rarely known to metastasize to lymph nodes. This is attributed to paucity of lymphatic channels in the bone. Even though some bone sarcomas like osteosarcoma are known to have nodal metastasis, such affections have not been reported with primary osseous chondrosarcoma. We describe a case of primary chondrosarcoma of proximal humerus with axillary nodal metastasis. The patient underwent a forequarter amputation with axillary nodal clearance. The histopathological examination revealed metastasis of chondrosarcoma in lymph nodes. We also have reviewed the related literature and discussed the possible mechanism of this nodal metastasis.

Enchondroma of the hand: the role of biopsy in the course of diagnosis and treatment

Enchondroma is the most frequent bone tumor of the hand, but chondrosarcoma is rare at this location. There is a high possibility of correct diagnosis of enchondroma and differentiating from its malignant counterpart by precise clinical and radiologic assessment without biopsy, a subject of debate in the literature. At the present study we substantially investigate this problem, in our patients. Case records, radiographs, and histology of 52 solitary enchondroma patients who underwent operation in our hospital between 1998 and 2010, were reviewed. Special attention paid to pre and post -op diagnoses, and compared with each other. Eighty-six percent of our patients were between the second to fourth decades of life, with a slight female predominance. In all, the primary diagnosis of enchondroma according to clinical presentation and radi-ographic appearance, supported by intraoperative gross appearance of tumor, and confirmed histologically by permanent section analysis. There was no mismatch between radiologic and histologic diagnosis. we concluded that correct diagnosis of enchondroma is almost always possible by precise clinical and radiographic assessment with no need for histologic confirmation before definitive treatment

Condrosarcoma mixoide de bajo grado en senos paranasales / Low grade myxoid chondrosarcoma of paranasal sinus

El condrosarcoma es un tumor maligno que raramente compromete los senos paranasales. Su crecimiento es lento pero muy agresivo localmente. El tratamiento de elección es la resección amplia en bloque para evitar la recidiva. Se reporta un caso de un condrosarcoma mixoide que compromete las cavidades paranasales en un niño quien fue tratado quirúrgicamente con un seguimiento de 5 años.

The chondrosarcoma is a malignant tumor that rarely involve the paranasal sinus. They have a slow growth but with relentless local progression. The therapy of choice is a wide en bloc resection to avoid recurrences. We report a case of myxoid chondrosarcoma involving the sinonasal cavity in a child treated surgically with a follow up of 5 years.

Condrosarcoma / Chondrosarcoma

Se realizó una revisión bibliográfica y actualización sobre el control de pacientes con condrosarcoma. Se analizaron aspectos importantes como clasificación, sexo, edad, localización, cuadro clínico, imagenología; con énfasis en los estudios gammagráficos de Morphey, anatomía patológica, diagnóstico diferencial, tratamiento quirúrgico y complicaciones. En el tratamiento quirúrgico se hizo referencia fundamentalmente a la utilización de diferentes técnicas basadas en su comportamiento histológico y radiológico. Por último se expusieron las complicaciones más importantes de este tumor que fueron la recurrencia y la metástasis, también se explicaron algunos factores relacionados.

A bibliographic review and an updating of the control of patients with chondrosarcoma were made. Important aspects such as classification, sex, localization, clinical picture, imaging with emphasis in Morphey's gammagraphic studies, pathological anatomy, differential diagnosis, surgical treatment and complications, were analyzed. In the surgical treatment, reference mainly was made to the utilization of the different techniques based on their histological and radiological behaviour. The most important complications of this tumor, which are recurrence and metastasis, were exposed, and some related factors were also explained.

Maffucci' s syndrome complicated by intracranial chondrosarcoma: two new illustrative cases

Maffucci's syndrome is a rare congenital condition, sometimes misdiagnosed as Ollier's disease, characterized by multiple enchondromas combined with hemangiomas and phlebectasia. Coexisting primary malignancies have been described sporadically. We report two cases of Maffucci's syndrome associated with cranial base chondrosarcoma, emphasizing pathophysiological features and the challenging management of intracranial chondrosarcomas. To the best of our knowledge, only twelve similar cases have been reported in the literature.

Síndrome de Maffucci é uma condição congênita rara, às vezes confundida com a doença de Ollier, caracterizada por encondromas múltiplos associados com hemangiomas e flebectasia. A concomitância com neoplasias primárias tem sido relatada esporadicamente. Nós relatamos dois casos de síndrome de Maffucci associada a condrossarcoma da base do crânio, enfatizando aspectos fisiopatológicos e o manejo desafiador dos condrossarcomas intracranianos. Em revisão da literatura, podemos encontrar o relato de apenas doze casos similares.

Condrosarcoma gigante de cartílago cricoides: informe de un caso / Giant chondrosarcoma of the cricoid cartilage: a case report

El condrosarcoma de laringe es un tumor raro que se localiza fundamentalmente en el cartílago cricoides. El objetivo de este artículo fue la presentación de un paciente con un condrosarcoma gigante de cartílago cricoides, que fue diagnosticado y tratado en el Servicio de Cirugía General del Hospital Hermanos Ameijeiras. Se describen los principales aspectos de la presentación clínica de la enfermedad, así como el resultado de los estudios de diagnóstico y la técnica quirúrgica empleada(AU)

Laryngeal chondrosarcoma is a rare tumor mainly located in the cricoid cartilage. The objective of this article was to present the case of a patient with giant chondrosarcoma of the cricoid cartilage diagnosed and treated at Hermanos Amejeiras hospital general surgery service. The main aspects of the clinical presentation of this disease together with the results of diagnosing studies and the surgical technique performed in this case were described(AU)

Condrossarcoma primário de língua em cão / Primary chondrosarcoma in the tongue of a dog

A 10 year old female Boxer was taken dead to veterinary hospital for a necropsy without any clinical history, showing a growth on the dorsal part of the tongue. Based on the histopathological findings mesenchymal chondrosarcoma was diagnosed. This report describes the first case of chondrosarcoma on the base of the tongue in veterinary medicine.

Laryngeal chondrometaplasia: a great mimic of chondrosarcoma.

Chondrometaplasia of larynx is a rare entity with an unknown etiopathogenesis. The lesion remains asymptomatic unless it reaches a large size and a history of trauma is usually elicited. It is a known pitfall in diagnostic evaluation and a clinically significant differential considered in evaluation of cartilaginous tumors of the larynx. A 66-year-old man presented with a nodular mass of right side of neck, progressive hoarseness of voice and pain, and a suspicious growth in the right glottic region. A CT followed by an MRI one month later revealed a slow growing ill defined thickening and enhancement of the supraglottic soft tissues, predominantly involving the submucosa, along the greater cornu on the left side of the thyroid cartilage, with extra-chondral enhancement and was suggested to be of infectious etiology. On neck exploration a lesion clinically suspected to be a chondrosarcoma was excised along with the right ala of the thyroid cartilage. The tissue processed entirely, revealed features consistent with chondrometaplasia of larynx. The importance of distinguishing this lesion from cartilaginous tumors is emphasized.

Estudo da expressão imunoistoquímica das proteínas não-colágenas da matriz extracelular óssea e dos fatores de transcrição CBFA1 e sox9 em osteossarcomas condrossarcomas de maxila e mandíbula / Study of the immunohistochemical expression of bone extracelular matriz non-colagen proteins and transcription factores Cbfa1 and Sox9 in jawbones osteosarcoma and chondrosarcomas

Osteossarcoma e condrossarcoma de maxila e mandíbula podem apresentar aspecto histopatológico semelhaente o que dificulta o diagnóstico diferencial. O objetivo deste trabalho foi estudar a expressão imunoistoquímica das principais proteínas não-colágenas da matriz extracelular óssea, a saber osteonectina (ONC), osteopontina (OPN), sialoproteina óssea (BSP) e osteocalcina (OCC), e dos fatores de transcrição Cbfa1 e Sox9 em 42 casos de osteossarcoma e 6 de condrossarcoma...

Myxoid Chondrosarcoma of the Sinonasal Cavity in a Child: a Case Report

Chondrosarcomas are malignant tumors of cartilage that rarely involve the sinonasal region, and myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma that usually occurs in the soft tissue of extremities. Although several case reports and results of small series of chondrosarcomas in the sinonasal region in children are available, myxoid type chondrosarcoma is extremely rare. We recently experienced a case of low grade myxoid chondrosarcoma involving the sinonasal cavity in a 10-year-old boy, and here we report its radiologic-pathologic findings. In this case, chondroid calcification on CT and septal and marginal enhancement on MRI suggested a chondrosarcoma. Whole body PET-CT demonstrated no definite metastatic lesion and a low peak standardized uptake value primary tumor. However, no definite distinguishing imaging features were observed that distinguished low grade myxoid chondrosarcoma from conventional chondrosarcoma.

Condrossarcoma em paciente com osteocondromatose múltipla: relato de caso e revisão da literatura / Chondrosarcoma in a patient with multiple osteochondromatosis: a case report and review of the literature

Os autores relatam um caso de condrossarcoma em uma paciente de 14 anos portadora de osteocondromatose múltipla, que procurou assistência médica com a queixa de tumoração na coxa esquerda. Cortes tomográficos do membro afetado evidenciaram lesão osteodestrutiva no terço superior do fêmur esquerdo. O diagnóstico foi feito através da revisão da lâmina do serviço de origem da paciente, confirmando o achado de condrossarcoma grau I em lesão condromatosa. Foi realizada desarticulação do colo femoral devido ao extenso comprometimento de grupos musculares, de tecido celular subcutâneo e de pele. A literatura mostra ampla variação nas taxas de transformação maligna das exostoses em pacientes portadores desta doença, sendo esta uma importante complicação nestes pacientes.

The authors report a case of chondrosarcoma in a 14-year-old female patient with multiple osteochondromatosis who has sought medical assistance complaining of a tumor on the left coxa. CT images of the affected limb have demonstrated osteodestructive lesion on the superior third of the left femur. The diagnosis has been made through a review of the biopsy slide from the service of origin of the patient, confirming the finding of a grade I chondrosarcoma in a chondromatous lesion. Disarticulation of the femoral neck was made because of the large extent of the lesion in muscular groups, subcutaneous cellular tissue and skin. The literature shows a wide variation in rates of malignant transformation of exostosis in patients with this disease, and this is a significant complication in patients affected by this disease.