Differential diagnosis and surgical management in chondrosarcoma of the jugular foramen / 中华耳鼻咽喉头颈外科杂志

Objective: To explore the diagnosis, surgical management and outcome of jugular foramen chondrosarcoma (CSA). Methods: Fifteen patients with jugular foramen CSA hospitalized in the Department of Otorhinolaryngology Head and Neck Surgery of Chinese PLA General Hospital from December 2002 to February 2020 were retrospectively collected,of whom 2 were male and 13 were female, aging from 22 to 61 years old. The clinical symptoms and signs, imaging features, differential diagnosis, surgical approaches, function of facial nerve and cranial nerves IX to XII, and surgical outcomes were analyzed. Results: Patients with jugular foramen CSA mainly presented with facial paralysis, hearing loss, hoarseness, cough, tinnitus and local mass. Computed tomography (CT) and magnetic resonance (MR) could provide important information for diagnosis. CT showed irregular destruction on bone margin of the jugular foramen. MR demonstrated iso or hypointense on T1WI, hyperintense on T2WI and heterogeneous contrast-enhancement. Surgical approaches were chosen upon the sizes and scopes of the tumors. Inferior temporal fossa A approach was adopted in 12 cases, inferior temporal fossa B approach in 2 cases and mastoid combined parotid approach in 1 case. Five patients with facial nerve involved received great auricular nerve graft. The House Brackmann (H-B) grading scale was used to evaluate the facial nerve function. Preoperative facial nerve function ranked grade Ⅴ in 4 cases and grade Ⅵ in 1 case. Postoperative facial nerve function improved to grade Ⅲ in 2 cases and grade Ⅵ in 3 cases. Five patients presented with cranial nerves Ⅸ and Ⅹ palsies. Hoarseness and cough of 2 cases improved after operation, while the other 3 cases did not. All the patients were diagnosed CSA by histopathology and immunohistochemistry, with immunohistochemical staining showing vimentin and S-100 positive, but cytokeratin negative in tumor cells. All patients survived during 28 to 234 months' follow-up. Two patients suffered from tumor recurrence 7 years after surgery and received revision surgery. No complications such as cerebrospinal fluid leakage and intracranial infection occurred after operation. Conclusions: Jugular foramen CSA lacks characteristic symptoms or signs. Imaging is helpful to differential diagnosis. Surgery is the primary treatment of jugular foramen CSA. Patients with facial paralysis should receive surgery in time as to restore the facial nerve. Long-term follow-up is necessary after surgery in case of recurrence.

Diagnosis and treatment of rare malignant temporal bone tumors / 临床耳鼻咽喉头颈外科杂志

Objective:To analyze the diagnosis, treatment and prognosis of patients with rare malignant tumors of the temporal bone. Methods:Four cases of rare temporal bone malignant tumors in our hospital between March 2014 and December 2020 were reviewed, including two cases of chondrosarcoma, one case of fibrosarcoma and one case of endolymphatic cystic papillary adenocarcinoma. There were three males and one female, ages between 28 and 56 years at the time of surgery. Common symptoms included hearing loss, facioplegia, tinnitus, and headache. All patients underwent imaging examinations to evaluate the extent of the lesions. Tumors were removed by subtotal temporal bone resection or infratemporal fossa approach, and postoperative adjuvant radiotherapy was applied if necessary. Results:One of the two chondrosarcoma patients was cured by complete resection of the tumor for 75 months, the other one recurred after the first excision of the tumor and underwent infratemporal fossa approach resection of skull base mass again with no recurrence found yet for 112 months. One patient with fibrosarcoma survived for 28 months after surgery with a positive margin and post-operative radiotherapy. One patient with endolymphatic cystic papillary adenocarcinoma recurred 12 months after subtotal lithotomy, and underwent subtotal temporal bone resection again, combined with radiotherapy. No recurrence was found for 63 months. Conclusion:The incidence of rare temporal bone malignant tumors is extremely low, the location is hidden, and the symptoms are atypical. Attention should be paid for early detection and early treatment. Surgical resection is the main treatment, and radiotherapy can be supplemented in the advanced stage or with a positive margin.

Tratamiento multidisciplinario de condrosarcoma gigante de fémur proximal mediante hemipelvectomía externa y reconstrucción con colgajo pediculado tipo fillet flap: presentación de un caso clínico / Multidisciplinary treatment of giant chondrosarcoma of the proximal femur by external hemipelvectomy and reconstruction with a pedunculated fillet flap: presentation of a clinical case / Tratamento multidisciplinar do condrossarcoma gigante do fêmur proximal por hemipelvectomia externa e reconstrução com retalho fillet flap: apresentação de um caso clínico

El condrosarcoma forma parte de los tumores primarios malignos más frecuentes. Las localizaciones pélvicas y de raíz de muslo requerirán abordajes amplios con la consecuente dificultad de cobertura de partes blandas. La hemipelvectomía externa es habitualmente el procedimiento de elección para estas localizaciones. Implica la resección de la extremidad inferior en bloque asociada a la hemipelvis ipsilateral, dando como resultado un gran defecto de cobertura. En algunas circunstancias, la extensa resección de partes blandas hace imposible la utilización de colgajos rotacionales locales. La reconstrucción con colgajo tipo "Fillet flaps" ofrece a estos defectos de partes blandas la opción de lograr una adecuada cobertura. El objetivo del trabajo es mostrar la resolución de un caso poco convencional de condrosarcoma de fémur proximal y su reconstrucción de partes blandas con un método nunca antes utilizado en nuestro medio.

Chondrosarcoma is one of the most frequent malignant primary tumors. Pelvic and proximal femur locations require extensive approaches with the consequent difficulty of soft tissue reconstruction. External hemipelvectomy is usually the procedure for these locations. It involves total en bloc resection of the lower extremity associated with ipsilateral hemipelvis, resulting in a large coverage defect. In some cases, local rotational flaps are impossible. Reconstruction with fillet flaps offers an adequate coverage for soft tissue defects. The objective of this study is to show the resolution of an unconventional case of chondrosarcoma of the proximal femur and its soft tissue reconstruction with a method never before used in our country.

O condrossarcoma é um dos tumores primários malignos mais frequentes. As localizações das raízes pélvicas e da coxa exigirão amplas abordagens com a conseqüente dificuldade em cobrir os tecidos moles. A hemipelvectomia externa costuma ser o procedimento de escolha para esses locais. Envolve a ressecção do membro inferior em bloco associado à hemipelve ipsilateral, resultando em um grande defeito de cobertura. Em algumas circunstâncias, a ressecção extensa de partes moles impossibilita o uso de retalhos rotacionais locais. A reconstrução com retalhos de filé oferece a esses defeitos de tecidos moles a opção de obter uma cobertura adequada. O objetivo deste trabalho é mostrar a resolução de um caso não convencional de condrossarcoma do fêmur proximal e sua reconstrução de partes moles com método nunca antes utilizado em nosso meio.

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)

Giant chondrosarcoma of the chest wall: a rare surgical challenge

The chest wall chondrosarcoma (CWC) is a rare slowly growing primary tumor of the chest wall with an incidence of <0.5 per million person-years. We present the case of a giant CWC that caused a mass effect on the mediastinum, heart, and lung. Large tumors with thoracic structures compression may be life threatening, and its resection and subsequent chest wall reconstruction represent a significant multidisciplinary surgical challenge. In this case, despite the large tumor dimensions, the preoperative planning—sparing key reconstructive options without compromising the tumor resection—allowed a complete en bloc tumor excision of a grade III chondrosarcoma with negative histologic margins. Successful reconstruction of the large full-thickness chest wall defect, with a latissimus dorsi muscle flap and methyl methacrylate incorporated into a polypropylene mesh in a sandwich fashion, was accomplished. Patient recovery was uneventful with good functional and aesthetic outcomes, and no evidence of recurrence at 1.5 years follow-up. This case report illustrates the main clinical, radiological, and histologic features of a CWC while discussing the surgical goals and highlighting the principles for chest wall reconstruction following extensive resection of a large and rare entity.

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)

Doble primario sincrónico: condrosarcoma y sarcoma fusocelular de alto grado asociado a síndrome de Maffucci / Synchronous primary double: condrosarcoma and sarcoma high grade fusocelular associated with Maffucci syndrome

Resumen: El síndrome de Maffucci se caracteriza por la presencia de múltiples encondromas y hemangiomas que pueden afectar tejidos blandos y otros órganos. El riesgo de transformación maligna de las lesiones es de 100% durante la vida del individuo, siendo el condrosarcoma el tumor maligno más frecuentemente asociado. Se presenta el caso de un hombre de 44 años de edad con diagnóstico de síndrome de Maffucci, el cual desarrolló un doble primario sincrónico: condrosarcoma y sarcoma fusocelular de alto grado multicéntrico de región escapular y tricipital, fue tratado con desarticulación interescapulotorácica, mostró progresión acelerada y enfermedad pulmonar. Existen otras neoplasias asociadas al síndrome de Maffucci tales como adenocarcinoma de páncreas, tumores mesenquimales de ovario, gliomas, astrocitomas y tumores de la pituitaria. Por lo que resulta muy interesante reportar la asociación infrecuente entre un sarcoma fusocelular y un condrosarcoma secundario en pacientes con síndrome de Maffucci. El seguimiento en este grupo de pacientes es complejo y se basa en la búsqueda intencionada de lesiones con crecimiento acelerado, prestando atención en lesiones de crecimiento progresivo, síntomas clínicos o datos radiológicos de malignidad.

Abstract: Maffucci syndrome is characterized by the presence of multiple enchondromes and hemangiomas that can affect soft tissues and other organs. The risk of malignant transformation of lesions is 100% during the life of the individual, with chondrosarcoma being the most frequently associated malignant tumor. We present the case of a 44-year-old man diagnosed with Maffucci syndrome who developed a synchronous double primary: chondrosarcoma and high-grade multicenter fusocellular sarcoma of scapular and tricipital region, was treated with disarticulation interscapule-thoracic, presented accelerated progression and lung disease. There are other neoplasms associated with Maffucci syndrome, such as pancreatic adenocarcinoma, mesenchymal ovarian tumors, gliomas, astrocytomas and pituitary tumors. It is therefore very interesting to report the uncommon association between fusocellular sarcoma and secondary chondrosarcoma in patients with Maffucci syndrome. Follow-up in this group of patients is complex and is based on the intentional search for accelerated growing lesions, paying attention to progressive growth injuries, clinical symptoms or radiological malignancy data.

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.

Sacrectomía parcial por abordaje posterior único / Partial sacrectomy by single posterior approach

Introducción: Los tumores del sacro representan <7% de los tumores espinales, prevalecen los tumores secundarios por mieloma múltiple o carcinomas de próstata, mama, pulmón o colón. El cordoma es el tumor maligno primario más frecuente y el tumor de células gigantes es la lesión benigna más común. Por su evolución, compromiso de estructuras extraóseas y la escasa respuesta a los tratamientos coadyuvantes, la cirugía es el tratamiento más utilizado, la vía de abordaje y la necesidad de instrumentación dependerán del tumor por tratar. Los objetivos de este trabajo son: evaluar el uso de la resección parcial del sacro, analizar la técnica quirúrgica y reconocer las complicaciones. Materiales y Métodos: Cuatro pacientes con diagnóstico de tumor sacro ubicado por debajo de S1, que consultan por dolor y cuyas imágenes confirman la lesión. A todos se les realiza una resección en bloque por vía posterior preservando S1. Se describe la técnica. Resultados: Los estudios anatomopatológicos revelaron: un cordoma, un tumor maligno de vaina nerviosa, un condrosarcoma y una metástasis de carcinoma prostático. Se preservó la función de S1 en todos los pacientes; uno tiene disfunción vesical permanente. Se observaron una dehiscencia de la herida, una infección y una fístula de líquido cefalorraquídeo. Todos permanecen sin la enfermedad tras un seguimiento de entre 6 y 24 meses. Conclusiones: La resección parcial del sacro por abordaje posterior único se puede indicar cuando la lesión compromete desde S2 hacia distal y no hay compromiso sacroilíaco. La preservación de raíces es de vital importancia para garantizar mejores resultados posoperatorios y una menor tasa de infección. Nivel de Evidencia: IV

Introduction: Tumors of the sacrum represent less than 7% of spinal tumors. Secondary tumors due to multiple myeloma or prostate, breast, lung and colon carcinomas predominate. Chordoma is the most frequent primary malignant tumor and giant cell tumor is the most common benign lesion. For its evolution, involvement of extra bone structures and poor response to the adyuvant therapy, surgery is the most commonly used treatment, the approach and the need for instrumentation depend on tumor to treat. The objectives of this study are to evaluate the use of partial resection of sacrum, analyze the surgical technique and assess complications. Methods: Four patients with diagnosis of sacral tumor below S1, who presented with pain and images confirming the injury. All underwent an en bloc resection by posterior via preserving S1. The technique is described. Results: The pathological results were: a chordoma, a malignant nerve sheath tumor, a chondrosarcoma and a metastasis of prostatic carcinoma. S1 function was preserved in all patients; one has permanent bladder dysfunction. Wound dehiscence, infection and cerebrospinal fluid fistula were detected. Patients are free of disease after a follow-up of 6-24 months. Conclusions: Partial resection of the sacrum using a unique posterior approach may be indicated when the injury involves from S2 distally and no sacroiliac involvement is observed. Root preservation is vital to guarantee the best postoperative results and a lower rate of infection. Level of Evidence: IV

Condrosarcoma de la primera vértebra cervical. Reporte de caso / Condrosarcoma of the first cervical vertebra. Case report

Fundamento: Los tumores primarios de la columna vertebral son raros, con una incidencia estimada de 2.8-8.5 por cada 100 000 individuos anualmente. El condrosarcoma es la segunda neoplasia ósea más frecuente de los tumores óseos primarios malignos, presentándose principalmente en el esqueleto axial: 45 %; siendo muy raros en los huesos craneofaciales y la columna. Objetivo: Describir la presentación clínica de un caso con un condrosarcoma en la primera vértebra cervical y la conducta quirúrgica tomada una vez diagnosticado Presentación de caso: Paciente masculino de 86 años de edad con antecedentes de hipertensión arterial controlada farmacológicamente, que comienza con cervicalgia de difícil tratamiento, tortícolis y disfagia a los sólidos. Se diagnostica mediante TAC de unión cráneo-espinal lesión tumoral en C1. Se le realiza exéresis parcial del tumor con instrumentación occipito-cervical, el paciente evoluciona satisfactoriamente. Conclusiones: El condrosarcoma es un tumor de crecimiento lento, de características variables. La localización espinal es muy rara. Su tratamiento incluye la resección de la lesión con instrumentación si se desestabiliza la columna vertebral, además de quimioterapia y radioterapia de ser necesario.

Background: The primary tumors of the spine are strange, with a dear incidence of 2.8-8.5 of each 100 000 individuals annually. The condrosarcoma is the second more frequent bony neoplasia of the wicked primary bony tumors, being presented mainly in the axial skeleton: 45%; being very strange in the bones craneofacials and the column. Objective: To describe the clinical presentation of a case with a condrosarcoma in the first cervical vertebra and the surgical behavior taken once diagnosed. Case presentation: 86 years old masculine patient with antecedents of pharmacologically controlled hypertension that it begins with cervicalgia of difficult treatment, torticollis and disfagia to the solids. It is diagnosed by means of TAC of union skull-spinal lesion tumoral in C1.a partial exeresis of the tumor with occipito-cervical instrumentation is carried out, the patient evolves satisfactorily. Conclusions: The condrosarcoma is a tumor of slow growth, of variable characteristics. The spinal localization is very strange. Their treatment includes the resection of the lesion with instrumentation if the spine is destabilized, besides chemotherapy and radiotherapy of being necessary.

Laryngeal chondrosarcoma - Ten years of experience / Condrossarcoma da laringe - 10 anos de casuística

INTRODUCTION: Laryngeal involvement by cartilaginous tumors is rare. However, although accounting for only 1% of laryngeal tumor pathology, they are the most frequently occurring non-epithelial neoplasms. The most probable location is the endolaryngeal surface of the cricoid cartilage. Their symptoms are variable, depending on the size and location, and may include hoarseness, stridor, and dyspnea. Treatment is based on surgical excision. Some centers take into account the degree of differentiation and whether it is a case of relapse when deciding to perform a radical resection. AIM: To evaluate this disease in a sample of the Portuguese population. METHODS: A review of the medical records from 2002 to 2012 by assessment of clinical processes was performed. Data on demographics, clinical treatments, and outcomes were collected. RESULTS: Six patients were included in the study. Five of them underwent total laryngectomy, and in one case, partial excision of the thyroid cartilage was performed. None of the patients had either metastases or tumor-related death. CONCLUSION: Laryngeal chondrosarcomas remain a rare disease of unknown etiology, with slow and insidious symptoms. The treatment is surgical, with favorable prognosis, and metastases rarely occur. The main concern regards their propensity to relapse. .

INTRODUÇÃO: O acometimento laríngeo por tumores cartilaginosos é raro. No entanto, apesar de representarem 1% da patologia tumoral laríngea, são as neoplasias não epiteliais mais frequentes. Localizam-se mais frequentemente na face endolaríngea da cartilagem cricóide. Tem sintomatologia variável consoante o tamanho e a localização, podendo incluir disfonia, estridor e dispneia. O tratamento é essencialmente cirúrgico. Alguns centros levam em conta o grau de diferenciação e de se tratar ou não de recidiva, quando da decisão de ressecção mais ou menos radical. OBJETIVO: Avaliar esta patologia numa amostra da população portuguesa. MÉTODO: Revisão da casuística no intervalo de tempo 2002-2012, através de consulta dos processos clínicos. Foram coligidos os dados demográficos e clínicos relevantes, os tratamentos efetuados e os resultados. RESULTADOS: Foram incluídos seis pacientes. Cinco foram submetidos à laringectomia total e um foi submetido à excisão da asa esquerda da cartilagem tiroide. Nenhum apresentou metástases ou morte relacionada com o tumor. CONCLUSÃO: Os condrossarcomas laríngeos permanecem como patologia rara, de etiologia desconhecida, com crescimento lento e clínica insidiosa. O tratamento é cirúrgico, com prognóstico favorável, com a metastização a ocorrer raramente. A maior preocupação decorre da sua propensão à recidiva. .

Condrosarcoma del septum nasal: reporte de un caso y revisión de la literatura / Chondrosarcoma of the nasal septum: a case report and literature review

El condrosarcoma del tabique nasal es una neoplasia poco frecuente. Cuando esto ocurre, el diagnóstico precoz es difícil porque los pacientes generalmente presentan síntomas rinosinusales inespecíficos y en forma tardía. Se presenta un caso clínico de una mujer de 37 años de edad que se presentó con una historia de exoftalmo sin síntomas rinosinusales agregados. El estudio imagenológico mostró una masa de aspecto neoplásico en el tabique nasal con extensión al seno maxilar, celdillas etmoidales, cavidad orbitaria y hacia posterior a la fosa pterigopalatina. Se consideró un tumor naso-orbitario por lo que se complementó estudio con nasofibroscopía flexible que mostró una masa nasal de aspecto liso, brillante, rosada que ocupaba piso, tabique y pared lateral de fosa nasal izquierda que no era separable del tabique nasal. Se realizó biopsia que mostró una histopatología sugestiva de condrosarcoma. Se realizó cirugía por abordaje endoscópico y con apoyo neuroquirúrgico por vía transcraneal. La presentación clínica, diagnóstico y tratamiento de este caso, así como una revisión de la literatura son discutidos.

Chondrosarcoma of the nasal septum is a rare malignancy. When this occurs, early diagnosis is difficult since patients usually present non specific symptoms rhinosinusal and late. There is a clinic case of a 37-year-old women who presented with a history of exophthalmos without added symptoms rhinosinusal. The image study showed a neoplasic mass appearance in the nasal septum with extension to the maxillary sinus, ethmoid cells, orbital cavity and post to the pterygopalatine fossa. We have considered a naso-orbital tumor which was complemented nasofibroscopy flexible nasal showed a pink mass of smooth, shiny, occupied floor, septum and lateral wall of left nasal cavity that was not separable from the nasal septum was considered. We have performed a biopsy wich shows a suggestive chondrosarcoma. Surgery was performed by endoscopic approach and via transcranial neurosurgical support. The clinical presentation, diagnosis and treatment of this case and a review of the literature are discussed.

Giant chondrosarcoma of chest wall.

Primary chest wall tumours are very rare. Chondrosarcoma is the most common tumour arising from the chest wall. We describe the occurrence of a slow-growing chondrosarcoma arising from the anterior chest wall in a 35-year-old male patient. The tumour was resected successfully and chest wall was reconstucted with prolene mesh and muscle flap. The patient was discharged uneventfully without any respiratory compromise. There was no recurrence after a three-year follow-up. Wide surgical resection with chest wall reconstruction appears to be the preferred treatment option for this rare tumour of the chest wall.

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 bone: does the size of the tumor, the presence of a pathologic fracture, or prior intervention have an impact on local control and survival?

AIMS AND OBJECTIVES: We studied 45 patients with chondrosarcoma, without metastasis at diagnosis, who were surgically treated between January 2000 and December 2004 to evaluate the risk factors associated with local recurrence and metastasis. MATERIALS AND METHODS: Fourteen (31%) patients had had some major prior intervention, either in the form of an open biopsy or a curettage / unplanned excision, before presenting to us. Eight patients had pathologic fractures at presentation. None of the patients received adjuvant chemotherapy or radiotherapy. The follow-up duration ranged from 8-75 months. All survivors had a minimum follow-up of 36 months (range 36-75 months). RESULTS: There were 11 grade 1 (24.5%), 23 grade 2 (51%), and 11 grade 3 (24.5%) chondrosarcomas. Thirty-two (71%) patients had tumors that were larger than 8 cm in the greatest dimension. Margins were adequate in 31 patients. Twenty-five patients had disease relapse; there were four local failures, nine distant failures, and 12 combined failures. At the time of the last review, 12 patients had died, 11 were alive with disease, and 22 were free of disease. The cumulative event-free survival was 44% and the overall survival was 73%. CONCLUSION: Grade of tumor, size of tumor, and adequacy of resection might be important predictors of outcome. Local recurrence is a prelude to distant metastasis and portends poor ultimate survival. The presence of a pathological fracture could indicate biologically aggressive disease, and limb salvage in these cases should be advised with caution. Even in cases where there has been a prior unplanned intervention, local control can be achieved by subsequent adequate resection.

Mandibular periosteal (juxtacortical) chondrosarcoma

Periosteal (juxtacortical) chondrosarcoma (PC) is a well-differentiated malignant cartilage-forming tumor arising from the external bone surface, especially in long bones. The therapy of choice is en-bloc resection and, in general, its prognosis is good. This paper reports a rare case of PC affecting the mandible of a 41-year-old man. The lesion presented as a slow-growing-painless swelling that lasted 2 months. Computed tomography scan showed a tumoral mass arising from the external bone surface, extending into the adjacent soft tissue presenting patchy regions of popcorn-like calcifications. A final diagnosis of PC (grade II) was rendered after biopsy. Hemimandibulectomy was undertaken followed by complementary radiotherapy with 70 Gy. Although no episodes of recurrence or metastasis had been noticed after 18 months of follow-up, the patient died and causa mortis could not be established.

Condrossarcoma (juxtacortical) periosteal (CP) é um tumor maligno produtor de cartilagem bem diferenciado que normalmente surge na superfície externa dos ossos longos. O tratamento de escolha para esse tumor é a ressecção em bloco e, em geral, apresenta um prognóstico bom. Nesse trabalho, nós apresentamos um caso raro de CP afetando a mandíbula de um homem de 41 anos de idade. A lesão apresentava um crescimento lento e indolor de 2 meses de evolução. A tomografia computadorizada revelou a presença de uma massa tumoral surgindo na superfície externa do osso mandibular exibindo agregados de tecido calcificado no seu interior e que se estendia para os tecidos moles adjacentes. Depois de realizada uma biópsia, um diagnóstico final de CP (grau II) foi estabelecido. O tratamento de escolha foi hemimandibulectomia seguida por radioterapia complementar na dose de 70 Gy. Embora nenhum episódio de recorrência ou metástase tenha sido noticiado após 18 meses de acompanhamento, o paciente morreu e a causa mortis não pôde ser determinada.

Condrossarcoma mixóide de átrio direito / Right atrium myxoid chondrosarcoma

Relatamos o caso de uma paciente de 46 anos, cor branca, hipertensa há 20 anos, com suspeita de endocardite infecciosa. Foi realizado ecocardiograma transtorácico, o qual levou à hipótese de mixoma de átrio direito. A paciente foi submetida à cirurgia, observando-se massa tumoral de aspecto muco-gelatinoso friável. A massa foi submetida a congelação para exame anatomopatológico, com laudo sugestivo de tumor maligno mesenquimal. Foi realizado exame imunohistoquímico compatível com condrossarcoma mixóide. A paciente evoluiu com remissão espontânea do quadro após ressecção completa da neoplasia. Realizou acompanhamento ambulatorial por 14 meses, sem apresentar sinais de recidiva do tumor.

We report a case of a 46 years-old woman, Caucasian, with hypertension and a primary dianosis of infectious endocarditis. A transthoracic echocardiogram was performed suggesting right atrium myxoma. The patient was submitted to surgery, which found a tumor mass with a jelly-like exterior. The mass was sent to anatomo-pathological analysis which diagnosed a myxoid chondrosarcoma tumor. After surgical resection, the patient achieved complete recovery with no signs of recidivation after 14 months.