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.

Condrosarcoma del hueso hioides: a propósito de un caso / Chondrosarcoma of the hyoid bone: a case report

Resumen Los condrosarcomas son cánceres realmente infrecuentes en cabeza y cuello, y más aún en el hueso hioides. Por lo general, son neoplasias que debutan como una masa cervical sin otra sintomatología. Su diagnóstico requiere de estudios de imagen y su tratamiento es fundamentalmente quirúrgico. Comentamos el caso de un paciente de 57 años, desde el diagnóstico de la lesión hasta su tratamiento y seguimiento, y una revisión bibliográfica de esta patología.

Abstract Chondrosarcomas are a rare type of head and neck cancer, especially in the hyoid bone. They usually make their debut through a cervical mass, without other symptoms. The diagnosis requires image studies, and the treatment is fundamentally surgical. We report the case of a 57 years old patient, from the lesion diagnosis, its treatment and follow up, and a bibliographic review of this pathology.

Cirugía de salvamento en un caso de condrosarcoma de rodilla / Salvage surgery in a case of knee chondrosarcoma

El siguiente caso, se trata de una paciente de sexo femenino que acude a consulta de traumatología por dolor de rodilla y dificultad para deambular, sin antecedente de trauma. En la radiografía anteroposterior y lateral se evidencia múltiples áreas radiolúcidas y escleróticas en meseta tibial y fémur distal derecho. La biopsia reporta: Condrosarcoma indiferenciado Grado 2. Se inicia sesiones de quimioterapia profiláctica y se planifica la cirugía de salvamento, esto ante la negativa de la paciente para aceptar la cirugía de amputación. La cirugía de reconstrucción se realizó mediante la colocación de una prótesis semiconstreñida tipo Endo Model cementada. En el posoperatorio se realizó controles y curaciones semanales, con buena cicatrización de la herida, y posteriormente rehabilitación por fisioterapia. Actualmente la paciente puede deambular con apoyo y casi de manera independiente.

The following case is a female patient who came to the trauma clinic for knee pain and difficulty walking, with no history of trauma.The anteroposterior and lateral radiograph shows multiple radiolucent and sclerotic areas on the tibial plateau and the right distal femur.The biopsy reports grade 2 undifferentiated chondrosarcoma. Prophylactic chemotherapy sessions are started and salvage surgery is planned, this given the patient's refusal to accept amputation surgery. Reconstruction surgery was performed by placing a semi-constrained, cemented endo Model type prosthesis. In the postoperative period, weekly controls and dressings were carried out, with good wound healing and later rehabilitation by physiotherapy. Actually the patient can walk with support and almost independently.

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)

A rare case of vulvar extraskeletal myxoid chondrosarcoma: mimics and diagnostic clues

Only 14 cases of extraskeletal myxoid chondrosarcoma (EMC) of the vulva have been documented in the literature. We report a case of a 63-year-old woman with EMC of the vulva confirmed by both EWSR1 and NR4A3 fluorescence in situ hybridization, the latter of which is a more specific probe for this entity. The unusual location of this tumor of prominent myxoid morphology gave rise to a wide differential diagnosis, which necessitated thorough histologic evaluation and confirmatory ancillary testing in the form of immunohistochemistry and cytogenetic studies. This article aims to review extraskeletal myxoid chondrosarcoma of the vulva and various diagnostic clues to help differentiate it from its histologic mimics. This is the fifth case of vulvar EMC in the literature with confirmation of a NR4A3 gene rearrangement.

Expression of amphiregulin in enchondromas and central chondrosarcomas

OBJECTIVES: The aim of this study was to evaluate the role of amphiregulin protein, an epidermal growth factor receptor ligand, in cartilaginous tumors. METHODS: Amphiregulin expression was examined in 31 enchondromas and 67 chondrosarcomas using immunohistochemistry analysis. RESULTS: Overall, 15 enchondromas (48.40%) and 24 chondrosarcomas (35.82%) were positive for amphiregulin. According to the receiver operating characteristic curve test, no difference in amphiregulin expression was observed between enchondromas and low-grade chondrosarcomas (p=0.0880). Additionally, 39 lesions (16 in short bones, 13 in long bones, and 10 in flat bones) were positive for amphiregulin, exhibiting a higher percentage of positive cells (p=0.0030) and intensity of immunohistochemical expression (p=0.0055) in short bone lesions than in others. Among 25 enchondromas localized in short bones, 15 expressed amphiregulin; however, all 6 cases localized in long bones were negative for this marker (p=0.0177). CONCLUSIONS: Amphiregulin did not help in distinguishing enchondromas from low-grade chondrosarcomas. The present study is the first to document the expression of this immunohistochemical marker in enchondromas. Furthermore, amphiregulin expression in enchondromas was localized in short bones, indicating a phenotypic distinction from that in long bones. This distinction may contribute to an improved understanding of the pathogenesis of these lesions.

¿Qué debe saber el neurocirujano sobre condrosarcoma espinal primario? Condrosarcoma espinal primario / What should the neurosurgeon know about primary spinal chondrosarcoma? Primary spinal chondrosarcoma

El condrosarcoma ocupa el tercer puesto dentro de las neoplasias óseas primarias, siendo la columna vertebral una localización inusual. Según su etiología se clasifican en condrosarcoma primario o secundario a lesiones subyacentes de tipo cartilaginosa como el encondroma u osteocondroma; siendo entre el 80-90% de bajo grado. Pueden presentarse en cualquier nivel de la columna vertebral, siendo más frecuente en la región torácica y cervical, comprometiendo los elementos posteriores de la vértebra, los cuerpos vertebrales o ambos, en un 40%, 15% y 45% respectivamente. El síntoma más común es el dolor localizado asociado a manifestaciones neurológicas. El método diagnóstico de elección es la biopsia por punción y el tratamiento se basa fundamentalmente en la resección quirúrgica

Chondrosarcoma occupies the third position within the primary bony neoplasia's, with an unusual location at the level of the spine. According to their etiology, they are classified as primary chondrosarcoma or secondary to underlying cartilaginous lesions such as the enchondroma or osteochondroma, being between 80-90% of low grade. They can occur at any level of the spine, being more frequent in the thoracic and cervical region, compromising the posterior elements of the vertebra, the vertebral body or both, by 40%, 15% and 45% respectively. The most common symptom is localized pain associated with neurological manifestations. The diagnostic method of choice is biopsy and treatment is based primarily on surgical resection.

Osteocondromatosis sinovial de rodilla: una causa infrecuente de gonalgia en edad pediátrica / Synovial chondromatosis of the knee. A rare cause of knee pain in pediatric age

La osteocondromatosis sinovial es una metaplasia benigna de la membrana sinovial que afecta a 1 de cada 100 000 personas, en su mayoría adultos, y es extremadamente infrecuente en edad pediátrica. Predomina en grandes articulaciones, sobre todo la rodilla, y la sintomatología es, por lo general, inespecífica. Dado que la radiografía simple no suele ser concluyente, se recurre a la resonancia magnética nuclear y a la tomografía axial computarizada para orientar el diagnóstico. Se expone el caso de una paciente de 10 años de edad con gonalgia y dismorfia en la patela izquierda de seis meses de evolución, con diagnóstico de osteocondromatosis sinovial. Se presenta el caso dado que se trata de una entidad muy rara en niños, pero que requiere un tratamiento quirúrgico precoz para evitar sus posibles complicaciones, como la destrucción articular progresiva o la malignización a condrosarcoma.

Synovial osteochondromatosis consists of a synovial metaplasia which affects 1 per 100 000 people. It is a very rare disease among children. It typically affects large joints of the body, especially the knee. Due to the lack of specificity of the signs and symptoms and X-Ray images, imaging tests such as nuclear magnetic resonance or computerized tomography are frequently needed for diagnosis.We report a case of a ten-year-old female patient with a six months history of pain and deformity of left patella which was diagnosed with synovial osteochondromatosis. This case highlights the importance of clinical suspicion, not only because it is an extremely rare disease in children, but also because it needs a surgical treatment as soon as possible in order to avoid consequences it might have in pediatric age, as joint destruction or malignization to chondrosarcoma.

Radiomics models based on non-enhanced MRI can differentiate chondrosarcoma from enchondroma / 南方医科大学学报

OBJECTIVE@#To develop and validate radiomics models based on non-enhanced magnetic resonance (MR) imaging for differentiating chondrosarcoma from enchondroma.@*METHODS@#We retrospectively evaluated a total of 68 patients (including 27 with chondrosarcoma and 41 with enchondroma), who were randomly divided into training group (=46) and validation group (=22). Radiomics features were extracted from TWI and TWI-FS sequences of the whole tumor by two radiologists independently and selected by Low Variance, Univariate feature selection, and least absolute shrinkage and selection operator (LASSO). Radiomics models were constructed by multivariate logistic regression analysis based on the features from TWI and TWI-FS sequences. The receiver-operating characteristics (ROC) curve and intraclass correlation coefficient (ICC) analyses of the radiomics models and conventional MR imaging were performed to determine their diagnostic accuracy.@*RESULTS@#The ICC value for interreader agreement of the radiomics features ranged from 0.779 to 0.923, which indicated good agreement. Ten and 11 features were selected from the TWI and TWI-FS sequences to construct radiomics models, respectively. The areas under the curve (AUCs) of TWI and TWI-FS models were 0.990 and 0.925 in training group and 0.915 and 0.855 in the validation group, respectively, showing no significant differences between the two sequence-based models (>0.05). In all the cases, the AUCs of the two radiomics models based on TWI and TWI-FS sequences and conventional MR imaging were 0.955, 0.901 and 0.569, respectively, demonstrating a significantly higher diagnostic accuracy of the two sequence-based radiomics models than conventional MR imaging (<0.01).@*CONCLUSIONS@#The radiomics models based on TWI and TWI-FS non-enhanced MR imaging can be used for the differentiation of chondrosarcoma from enchondroma.

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.

Chondrosarcoma original from the zygomatic arch: A case report and literature review / 中南大学学报(医学版)

Chondrosarcoma original from the zygomatic arch is a very rare disease with high malignancy. Surgery is the main means of treatment at present for duo to its poor sensitivity to radiochemotherapy. We reported a young patient who was recovery well in a 4-years follow-up without radiochemotherapy after a total resection of the tumor.

Clinical Significance of Resection Type and Margin following Surgical Treatment for Primary Sarcoma of the Spine: A Multi-Center Retrospective Study

STUDY DESIGN: A retrospective multi-center study.OBJECTIVES: To analyze oncological outcomes according to the resection type and surgical margin following surgical treatment for primary spinal sarcoma.SUMMARY OF LITERATURE REVIEW: Previous studies using registry databases have shown that surgery and negative margins were associated with improved survival for primary spinal sarcoma. However, few studies have comprehensively analyzed the clinical significance of the resection type and surgical margin for the oncological outcomes of this rare malignancy.MATERIALS AND METHODS: We retrospectively reviewed consecutive patients who underwent surgical resection for primary spinal sarcoma between 1997 and 2016 at two tertiary medical centers. Overall survival and the occurrence of local recurrence and distant metastasis were compared between the groups using Kaplan-Meier curve analysis and the log-rank test.RESULTS: Thirty-three patients (21 males,12 females) with a mean age of 45.1 years and a median follow-up of 36 months were included. There were 13 (39.4%) chondrosarcomas, 12 (36.4%) osteosarcomas, and eight different histological diagnoses. The cohort was categorized into four groups: 1) total en bloc resection with a negative margin (n=12; 36.4%), 2) total en bloc resection with a positive margin: (n=5; 15.2%), 3) total piecemeal resection (n=12; 36.4%), and 4) subtotal resection (n=4; 12.1%). Total en bloc resection with a negative margin was associated with improved overall survival (p=0.030) and less distant metastasis (p=0.025) and local recurrence (p=0.004).CONCLUSIONS: Achieving a negative margin through total en bloc resection, although technically demanding, improves oncological outcomes in primary spinal sarcoma.

The Clinical Outcome of Dedifferentiated Chondrosarcoma / 대한정형외과학회잡지

PURPOSE: A dedifferentiated chondrosarcoma is a rare lethal tumor characterized by a low grade chondrosarcoma juxtaposed with a high grade dedifferentiated sarcoma, such as osteosarcoma, fibrosarcoma. The aim of our study was to document the clinical manifestation and oncologic outcomes of a dedifferentiated chondrosarcoma. MATERIALS AND METHODS: This study identified 11 patients who were diagnosed and treated for dedifferentiated chondrosarcoma between January 2007 and December 2016. The identified cohort was then reviewed regarding age, sex, symptom onset, tumor location, magnetic resonance imagings (MRIs), surgical margin, and pathologic diagnosis. The time to local recurrence and/or metastasis, follow-up duration, and the patients' final status were analyzed. RESULTS: The patients were comprised of 7 males and 4 females with a mean age of 54 years (range, 33–80 years). The location of the tumor was in the femur in 6 cases, pelvis in 4 cases, and metatarsal in 1 case. The average tumor diameter was 12.7 cm (range, 6.0–26.1 cm). At the time of diagnosis, 2 patients showed pathologic fracture; 1 patient was Enecking stage IIA, 9 patients were stage IIB, and 1 patient was stage III. Eight patients were classified as a primary dedifferentiated chondrosarcoma and 3 patients were secondary. One of the primary lesions was misinterpreted initially as a low grade chondroid lesion by MRI and underwent curettage. Local recurrence occurred in 8 cases and distant metastasis occurred in 10 cases with a mean duration of 8 months (range, 2–23 months) and 7 months (range, 1–32 months), respectively. The three-year overall survival of patients with dedifferentiated chondrosarcoma was 18%, and 10 patients died due to disease progression. CONCLUSION: Dedifferentiated chondrosarcoma developed lung metastases in the early period of the clinical courses and the prognosis was dismal.

Estudio clínico y molecular en una familia con osteocondromatosis múltiple / Clinical and molecular study in a family with multiple osteochondromatosis

Resumen: Se presentan dos casos de una familia con diagnóstico de osteocondromatosis múltiple, el cual fue confirmado por estudio molecular con mutación sin sentido en heterocigosis c.1219C>T, (p.Gln407Stop) en el gen EXT1. En el primer caso, en un paciente se presentó deformidad de Madelung como hallazgo infrecuente y en el otro caso, condrosarcoma como complicación temida, resaltando la variación intrafamiliar, por lo que se recomienda la evaluación individual e interdisciplinaria. Además, ante una entidad genética debe brindarse el adecuado y oportuno asesoramiento genético familiar a todos sus integrantes.

Abstract: We present two cases of a family with the diagnosis of multiple osteochondromatosis, which was confirmed by molecular study with nonsense in heterozygosis mutation c.1219C>T, (p.Gln407Stop) in the EXT1 gene. In these cases, the Madelung deformity was presented in one patient as an uncommon finding and chondrosarcoma as a feared complication in the other case, highlighting intrafamilial variation, which is why individual and interdisciplinary evaluation is recommended. In addition, before a genetic entity should provide adequate and timely family genetic counseling to all its members.

Role of Tc-99m MDP Bone Scintigraphy in the evaluation of suspected Chondrosarcoma from OIiier Disease: A rare case repor

@# OIiier disease is a rare nonhereditary disorder characterized by multiple enchondromas in which malignant changes may occur. We report the case of a 15-year-old male with Ollier disease who presented with a large mass in the left upper arm for several months. Radiography revealed hugelytic mass in the proximal half to two-thrids of the left humerus. Bone scan showed irregularly increased tracer uptake in the head to mid shaft of the left humerus that is suggestive of malignant disease. Pathology analysis demonstrated proximal humeral chondrosarcoma, grade 1. In Ollier disease, bone scan may be used for monitoring the lessions with suspicion for malignant transpormation.