Benign extra skeletal chondroma of the tongue in a 16-year-old female

This meta-analysis aims to comprehensively assess the efficacy and safety of both patent ductus arteriosus (PDA) stents and surgical aortopulmonary shunt (APS) as the initial palliative measures in babies relying on ductal-dependent circulation. This review is essential to compare the outcome of relatively newer catheter-based PDA stent procedure with a surgical APS. By synthesizing existing literature, this review aims to provide insights to inform clinical practice and enhance patient care in this challenging clinical scenario. The methodology involved an extensive search of PubMed and Embase databases using specific keywords and terminology related to mortality, procedural outcomes, and postprocedural complications following PDA stent and APS in cyanotic congenital heart defects (CHD) patients. Six retrospective observational studies met the criteria, with 757 patients included. The analysis showed comparable mortality rates between PDA stents and APS. However, PDA stents were associated with decreased risks of mechanical circulatory support, postprocedural complications, and shorter hospital stays, mechanical ventilation, and intensive care unit stays compared to APS. Notably, patients with pulmonary atresia scheduled for biventricular surgery were more prevalent among those receiving PDA stents. In conclusion, while the risk of mortality is similar between PDA stents and APS, PDA stents offer advantages such as shorter hospital stays and reduced complications. Patient characteristics also vary, with a higher prevalence of intact ventricular septum among those receiving PDA stents.

Condrossarcoma gigante de corpo esternal: relato de caso / Giant chondrosarcoma of the sternal body: case report

Os tumores de parede torácicas são relativamente inusitados, representando 1 a 2% de todas as neoplasias, e cerca de 5% das neoplasias torácicas. Sarcomas são tumores raros e com apresentação heterogênea. São divididos em dois grandes grupos: tumores ósseos e de partes moles. A ocorrência na parede torácica é rara, representando 8% de todos os sarcomas. O tratamento de escolha é a ressecção com margens ampliadas e, quando ocorre a invasão de estruturas profundas, a toracectomia é indicada. A reconstrução do arcabouço ósseo é realizada com materiais de síntese, proporcionando estabilidade, e é seguida da reconstrução de partes moles com retalhos musculares, musculocutâneos ou locais ao acaso. Os tumores malignos primários da parede torácica correspondem a menos de 1% de todas as neoplasias e incluem grande variedade de lesões ósseas e de tecidos moles. Os condrossarcomas representam 20% dos tumores primários da parede torácica, sendo que 80% têm origem nas costelas e 20% no esterno.

Chest wall tumors are relatively uncommon, representing 1 to 2% of all neoplasms, and approximately 5% of thoracic neoplasms. Sarcomas are rare tumors with heterogeneous presentation. They are divided into two large groups: bone and soft tissue tumors. Occurrence in the chest wall is rare, representing 8% of all sarcomas. The treatment of choice is resection with wide margins and, when invasion of deep structures occurs, thoracotomy is indicated. The reconstruction of the bone framework is carried out with synthetic materials, providing stability, and is followed by the reconstruction of soft tissues with muscular, musculocutaneous, or random local flaps. Primary malignant tumors of the chest wall account for less than 1% of all neoplasms and include a wide variety of bone and soft tissue lesions. Chondrosarcomas represent 20% of primary tumors of the chest wall, with 80% originating in the ribs and 20% in the sternum.

Incidental finding of an atypical cartilaginous tumor in an adult female with recurrent patellar dislocation: Single-stage extended curettage using freezing nitrogen ethanol composite followed by Medial Patello-Femoral Ligament Reconstruction

@#Atypical cartilaginous tumor (ACT) refers to a low-grade cartilaginous neoplasm microscopically identical to grade 1 chondrosarcoma, affecting the appendicular skeleton. Treatment with intralesional curettage has been found to provide sufficient local control with less morbidity compared to wide resection. This is the first reported case of a simultaneous medial patello-femoral ligament (MPFL) reconstruction with extended curettage for ACT on the ipsilateral femur. A 45-year-old female presented with chronic recurrent patellar dislocation of the right knee. Magnetic resonance imaging revealed a tear of the MPFL, with an incidental epi-metaphyseal chondroid lesion. After biopsy confirmed an ACT, single-stage extended curettage using freezing nitrogen ethanol composite (FNEC) and MPFL reconstruction was performed, followed by augmentation with bone cement and a distal femoral plate. Currently, the patient is independently ambulatory, with full range of motion about the knee. Following histologic confirmation of an ACT in the setting of a concurrent MCL tear, a single-stage procedure to address both conditions is a viable option that can reduce complications associated with multiple surgeries. Extended curettage using FNEC has been shown to produce good short-term oncologic outcomes while maximizing function.

IMP3, CDK4, MDM2 and β-catenin expression in Enchondroma and Central Chondrosarcoma: Diagnostic and prognostic utility

Abstract Introduction The role of IMP3, CDK4, MDM2 and β-catenin proteins in Enchondroma and Central Chondrosarcoma is not totally understood. The aim of this study is to evaluate the immunoexpression of these proteins, associating histological grade, clinical data and prognosis to these tumors. Methods This is a retrospective-analytical study of 32 Enchondroma and 70 Central Chondrosarcoma. Results IMP3, CDK4, MDM2 and β-catenin expression was observed in 22.82 %, 13.82 %, 17.17 % and in 8.8 % of cases, respectively. All Enchondromas positive for these immunomarkers were located in short tubular bones. The positivity for these antibodies is directly proportional to Chondrosarcoma's histological grade increase. No difference was found between Enchondroma and Chondrosarcoma, Grade 1 for IMP3, CDK4 and ß-catenin positivity. Significant metastasis outcome was observed for IMP3, CDK4, MDM2 and death for MDM2 expression. Conclusion IMP3, CDK4, MDM2 and β-catenin expression in Enchondromas of short bones phenotypically characterizes these tumors. Their expression has not proven to be useful either as diagnostic markers of these neoplasms or in distinguishing between Enchondroma and Chondrosarcoma, Grade 1. The significant immunoexpression of IMP3, CDK4 and MDM2 in metastatic Chondrosarcoma and the lower survival in those with positivity for MDM2 suggest a possible association of these proteins with tumor aggressiveness.

Márgenes quirúrgicos como factor pronóstico del condrosarcoma de pelvis. Estudio de cohorte en una unidad de sarcomas / Surgical margins as prognostic factor in pelvis chondrosarcoma. Cohort study in a sarcoma unit

Resumen: Introducción: el condrosarcoma (CS), el segundo tumor maligno óseo primario más común, constituye aproximadamente una cuarta parte de todos los sarcomas óseos primarios. Los márgenes quirúrgicos en el condrosarcoma pélvico tienen un impacto directo como factor pronóstico, tanto en la supervivencia global como en la supervivencia libre de recurrencia de esta enfermedad. Objetivos: analizar el impacto de los márgenes quirúrgicos como factor pronóstico en el condrosarcoma de la pelvis. Material y métodos: cohorte de base de datos retrospectiva con seguimiento prospectivo de sarcomas de pacientes con diagnóstico de condrosarcoma primario de la pelvis que fueron sometidos a tratamiento quirúrgico. Se obtuvieron variables clínico-demográficas, se realizó un análisis descriptivo de cada variable y se contrastaron con las variables desenlace. Resultados: se incluyeron 17 pacientes, de los cuales nueve eran mujeres. La mediana de edad fue de 41 años (rango de 23 a 65 años). El tamaño promedio del tumor fue de 20.9 cm (rango de 5 a 46 cm). El margen quirúrgico promedio fue de 5.3 mm, variando entre 1 y 30 mm, con 58% de márgenes positivos. La supervivencia global promedio fue de 64 meses (rango de 7 a 108 meses). La distribución de la afectación pélvica fue: zona I en nueve pacientes (52.9%), zona II en dos (11.8%), combinación de zonas I-III en dos (11.8%), I+II en uno (5.9%), II+III en uno (5.9%), I-III más sacro en uno (5.9%) y I más sacro en uno (5.9%). Los grados tumorales se clasificaron en bajo en siete pacientes (41.2%), intermedio en siete (41.2%), alto en dos (11.8%) y desdiferenciado en uno (5.9%). Respecto al tipo de resección, 12 pacientes (70.6%) se sometieron a hemipelvectomía interna y cinco (29.4%) a hemipelvectomía externa. Se registró recurrencia en cinco casos (29.4%), metástasis en tres (17.6%) y mortalidad en cuatro (23.5%). Conclusiones: esta serie representa la cohorte más extensa reportada en Latinoamérica de condrosarcomas primarios de la pelvis. Se observó un pronóstico más favorable en pacientes con márgenes quirúrgicos superiores a 1 mm. La presencia de condrosarcoma en múltiples zonas pélvicas se asoció con un peor pronóstico oncológico. Además, se identificó una mayor incidencia de márgenes quirúrgicos positivos y tasas de recurrencia local en condrosarcomas de la pelvis en comparación con aquellos ubicados en las extremidades.

Abstract: Introduction: chondrosarcoma is the second most common primary malignant tumor, constitutes approximately one quarter of all primary bone sarcomas. Surgical margins in pelvic chondrosarcoma have a direct impact as a prognostic factor, both on overall survival and on recurrence-free survival of this disease. Objectives: to analyze the impact of surgical margins as a prognostic factor in pelvic chondrosarcoma. Material and methods: a retrospective database cohort with prospective follow-up of sarcomas in patients diagnosed with primary pelvic chondrosarcoma who underwent surgical treatment. Clinical-demographic variables were obtained, a descriptive analysis of each variable was performed, and these were contrasted with the outcome variables. Results: seventeen patients were included, of which nine were female. The median age was 41 years, ranging from 23 to 65 years. The average tumor size was 20.9 cm (range 5 to 46 cm). The average surgical margin was 5.3 mm, ranging from 1 to 30 mm, with 58% positive margins. The average overall survival was 64 months (range 7 to 108 months). The distribution of pelvic involvement was as follows: zone I in nine patients (52.9%), zone II in two (11.8%), a combination of zones I-III in two (11.8%), I+II in one (5.9%), II+III in one (5.9%), I-III plus sacrum in one (5.9%) and I plus sacrum in one (5.9%). Tumor grades were classified as low in seven patients (41.2%), intermediate in sven (41.2%), high in two (11.8%), and dedifferentiated in one (5.9%). Regarding the type of resection, 12 patients (70.6%) underwent internal hemipelvectomy and five (29.4%) external hemipelvectomy. Recurrence was recorded in five cases (29.4%), metastasis in three (17.6%), and mortality in four (23.5%). Conclusions: this series represents the largest cohort reported in Latin America of primary pelvic chondrosarcomas. A more favorable prognosis was observed in patients with surgical margins greater than 1 mm. The presence of chondrosarcoma in multiple pelvic zones was associated with a worse oncological prognosis. Additionally, a higher incidence of positive surgical margins and local recurrence rates were identified in pelvic chondrosarcomas compared to those located in the extremities.

Nasal chondrogenic tumors: Case reports of primary chondroma and recurrence of atypical cartilaginous tumor (chondrosarcoma grade I) with review of the Literature

Nasal chondromas and nasal atypical cartilaginous tumors (or chondrosarcoma grade 1) are very rare chondrogenic tumors. Approximately 150 cases of nasal chondroma and fewer than 50 cases of nasal atypical cartilaginous tumors have been described. We reported a case with nasal chondroma in the right ethmoid area and a case with a nasal atypical cartilaginous tumor of the anterior septum. No chondroma recurrence was noted for 17 years after endoscopic resection. Recurrence was noted for the nasal atypical cartilaginous tumor 36 months after wide surgical excision via midfacial degloving. Revision surgery was performed again via midfacial degloving. Recurrences of nasal chondroma and atypical cartilaginous tumors are extremely rare. The current data indicate adequate surgical excision, in some cases even radical, as the most important parameter to avoid recurrence. For inoperable recurrent cases of atypical cartilaginous tumors, observation may be followed.

Extraskeletal mesenchymal chondrosarcoma of the abdomen: A rare clinical presentation

Extraskeletal mesenchymal chondrosarcoma (EMCS) is a rare malignant soft tissue tumor of chondroprogenitor cell origin. Originally, it was restricted to the bone only but that is no longer the case. Recent literature reports that 20–33% of these tumors occur at the extraskeletal sites. We report one such case, in which the tumor involved the anterior abdominal wall muscles and also had a large intra-abdominal mass that covered a large part of the peritoneal cavity. The clinical features and computed tomography findings suggested the diagnosis of a malignant desmoid tumor with intra-abdominal extension; however, the histopathological examination and the immunohistochemistry proved the tumor to be EMCS. The case is reported due to the dilemma in diagnosis, its rarity, large size, parietal, and intra-abdominal extension with multiple site involvement.

Challenges with Adjuvant Radiation for Intracranial Chondrosarcoma in Pregnancy

Background: Managing a brain tumor during pregnancy is a highly confusing and challenging situation, complicated by several technical, medical, ethical, and sociocultural concerns. The interests of the mother and child are often pitted against each other, for which legal opinion may occasionally be needed. Case Report?We present the report of a young lady with intracranial well-differentiated chondrosarcoma who was determined to be pregnant in the immediate postoperative period. We discuss the management of challenges and dilemmas in devising optimum therapy, and the modifications and care required at each step to help safeguard maternal and fetal health. Risks with therapeutic radiation and measures to assess and pre-empt fetal doses that may assist decision-making are also discussed. Conclusion?Radiation therapy during pregnancy is challenging and requires multidisciplinary involvement and psychosocial support for the patient and family.

Immunohistochemical analysis by ki67 and idh1 in patients with chondrosarcoma / Análise imuno-histoquímica por meio de ki67 e idh1 em pacientes com condrossarcoma

ABSTRACT Objective: To perform an immunohistochemical evaluation using the IDH1 and Ki67 markers in patients who underwent treatment for chondrosarcoma in a reference service center in Brazil. Methods: Retrospective analytical observational study using medical records of patients diagnosed with chondrosarcoma. Besides the epidemiological and clinical profile, important variables for prognosis and correlation with immunohistochemical analysis results with Ki67 and IDH1 markers were evaluated. Results: Histopathological examinations by immunohistochemistry of 56 patients were analyzed, 52% of which were women, with the age group 20-60 years being more prevalent. Grade 1 and 2 histological subtypes corresponded to most chondrosarcomas. The femur, humerus, and tibia were the most frequent anatomical sites. Most tumors (59%) were larger than 8 cm. Ki67 expression was very low (< 10%) in 98% of patients. The analysis of IDH1 was positive in 43% of the cases. The correlation between IDH1 positivity and tumor size was statistically significant, but regarding survival, we observed no significance. Conclusion: Immunohistochemical analysis using IDH1 and Ki67 markers in patients with conventional chondrosarcoma is not useful for prognostic guidance. Level of Evidence II, Prognostic Assessment, Results of Immunohistochemical Tests and Correlation with Survival.

RESUMO Objetivo: Fazer uma avaliação imuno-histoquímica usando os marcadores IDH1 e Ki67 em pacientes que fizeram tratamento para condrossarcoma em um serviço de referência no Brasil. Métodos: Estudo retrospectivo, analítico e observacional de prontuários de pacientes com diagnóstico de condrossarcoma. Além do perfil epidemiológico e clínico, foram avaliadas variáveis importantes para o prognóstico e a correlação com os resultados da análise imuno-histoquímica utilizando os marcadores Ki67 e IDH1. Resultados: Foram analisados exames histopatológicos por imuno-histoquímica de 56 pacientes, dos quais 52% eram do sexo feminino. A faixa etária mais prevalente foi entre 20 e 60 anos. Os subtipos histológicos graus 1 e 2 corresponderam à maioria dos casos. Fêmur, úmero e tíbia foram os sítios anatômicos mais frequentes. A maioria dos tumores (59%) tinha tamanho superior a 8 cm. O Ki67 teve expressão muito baixa (< 10%) em 98% dos pacientes. Já a análise do IDH1 foi positiva em 43% dos casos. A correlação entre a positividade do IDH1 e o tamanho do tumor foi estatisticamente significativa; já em relação à sobrevida, não houve significância. Conclusão: A análise imuno-histoquímica por meio dos marcadores IDH1 e Ki67 em pacientes com condrossarcoma convencional não é útil para orientação prognóstica. Nível de Evidência II, Avaliação Prognóstica, Resultados de Exames Imuno-Histoquímicos e Correlação com Sobrevida.

Comportamiento del condrosarcoma óseo en pacientes menores de 40 años / Chondrosarcoma of bone in young patients

Resumen El condrosarcoma ocupa el segundo lugar en frecuencia, entre los tumores malignos primarios óseos, en pacientes adultos. Típicamente afecta a adultos de entre 40 y 70 años, siendo la mayoría de las series publicadas de este grupo etario. El objetivo de este estudio fue comunicar una serie de pacientes adultos jóvenes (16 a 40 años) con diagnóstico de condrosarcoma óseo, que buscó describir las particularida des en la forma de presentación en esta población. De un total de 37 pacientes de 16 a 40 años operados por condrosarcoma en nuestro centro, entre los años 2008 y 2019, 18 presentaron tumores de bajo grado (G1/tumor cartilaginoso atípico), 16 de grado intermedio (G2), quedando una minoría con tumores de alto grado (2 con condrosarcoma G3 y 1 con un condrosarcoma desdiferenciado). La tasa de supervivencia global fue del 97% (IC 95% 82%-99%) a 2 años y 90.5% (IC 95% 73%-96%) a 5 años y la tasa libre de recurrencia local fue de 92% (IC 95% 77%-97.5%) a los 12 meses y de 89% a los 2 y 5 años (IC 95% 73%-96%). El grado histológico fue el factor pronóstico más relevante. La concordancia del grado histológico entre el diagnóstico preoperatorio de la biopsia y el material de resección quirúrgica fue del 81% (30/37).

Abstract Chondrosarcoma is the second most frequent primary malignant bone tumor. It is typically described in adults between 40 and 70 years of age, being the majority of the series published in this age group. The objective of this study was to report a series of young adults (16-40 years old) with a diagnosis of chondrosarcoma of bone in order to describe the particularities of this tumor in this population. From a total of 37 patients between 16 and 40 years old, surgically treated for chondrosar coma in our institution, 18 had low-grade tumors (G1/atypical cartilaginous tumor), 16 had an intermediate-grade (G2), and a minority had high-grade tumors (2 with a G3 and 1 with a dedifferentiated chondrosarcoma). The overall survival rate was 97% (95% CI 82%-99%) at 2 years of follow-up and 90.5% (95% CI 73-96) at 5 years of follow-up. The local recurrence free rate was 92% (95% CI % 77%-97.5%) at 1 year of follow-up and 89% at 2 and 5 years of follow-up (95% CI 73%-96%). Histological grade was the most relevant prognostic factor. The concordance of the histological grade between the preoperative diagnosis of the biopsy and the surgical resection material was 81% (30/37).

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.

Clear cell variant of chondrosarcoma of calcaneum: A case report with review of the literature

Clear cell chondrosarcoma (CCC) is an uncommon variant constituting less than 2% of all chondrosarcomas. CCC arises most commonly in the proximal end of the femur, followed by the humerus, and other small bones. CCC involving the tarsal bone is very rare, and to the best of our knowledge, only one case has been described involving the calcaneum in the English literature to date. In this article, we discuss a case of CCC involving the right calcaneum with complete clinico-immuno-histomorphological features, which is successfully managed by below-knee amputation. We also reviewed the reported cases of chondrosarcomas involving the calcaneum with special reference to clinical features, therapy, morphology, and follow-up data.

Primary renal mesenchymal chondrosarcoma: a case report / 中华泌尿外科杂志

Renal mesenchymal chondrosarcoma is a rare malignant soft tissue tumor. This paper reports a patient who was admitted to hospital because of left renal tumor, and underwent robot-assisted laparoscopic partial nephrectomy. The patient was diagnosed as left renal mesenchymal chondrosarcoma by pathological and genetic examination after operation. There was no local recurrence and metastasis six months after operation.

Clear cell chondrosarcoma of the proximal tibia - case report

ABSTRACT Introduction: Clear cell chondrosarcomas are a rare type of low-grade chondrosarcoma. Unlike conventional chondrosarcomas, these tumors occur mainly in the epiphyses of long bones, especially in the proximal femur and proximal humerus. Given their lytic characteristics with a cystic appearance, they can be mistaken for bone cysts and diagnosed late. Diagnosis must be made based on histopa-thology and immunohistochemistry. Likewise, negativity for cytokeratins and anti-endomysial antibodies are tools to rule out the differential diagnosis of metastasis. The treatment of this low-grade chondral lesion is surgical, either with intralesional management or complete resection. Case presentation: This is the case of a 46-year-old woman who consulted the orthopedic oncology service of a quaternary care institution due to medial knee pain at the level of the proximal tibia for 8 months. Diagnostic imaging studies showed a cystic lesion in the lateral tibial plateau; a biopsy of the lesion was performed, and clear cell chondrosarcoma was diagnosed based on histopathology and immunohistochemistry. Surgical management included tumor-free resection margins and reconstruction of the bone defect with a structural allograft of the proximal tibia. The patient progressed satisfactorily. Conclusions: This was the case of a patient with a subtype of low-grade chondrosarcoma that is rare in incidence and location, who was diagnosed based on immunohistochemistry and treated surgically to achieve a successful limb salvage.

RESUMEN Introducción. El condrosarcoma de células claras es un subtipo de tumor poco frecuente del grupo de los condrosarcomas de bajo grado. A diferencia de los condrosarcomas convencionales, estos tumores se presentan principalmente en epífisis de huesos largos. Dadas sus características líticas de aspecto quístico, pueden confundirse con quistes óseos, por lo que su diagnóstico debe hacerse por histopatología e inmunohistoquímica mediante biopsia. Igualmente, la negatividad para citoqueratinas y anticuerpos antiendomisio es una herramienta para descartar el diagnóstico diferencial de metástasis. Su tratamiento es quirúrgico, bien sea con manejo intralesional o resección completa con márgenes libres. Presentación del caso. Mujer de 46 años quien consultó al servicio de ortopedia oncológica de una institución de IV nivel de atención por un cuadro clínico de 8 meses de evolución consistente en dolor en la cara medial de la rodilla izquierda a nivel de la tibia proximaluna. Se practicaron imágenes diagnósticas que mostraron una lesión quística en el platillo tibial lateral; se ordenó biopsia de la lesión, y mediante histopatología e inmunohistoquímica se diagnosticó condrosarcoma de células claras. Se realizó un manejo quirúrgico con resección de la lesión con márgenes libres y una reconstrucción del defecto óseo con un aloinjerto estructural de tibia proximal, con lo cual se obtuvo una evolución satisfactoria. Conclusiones. Se presenta el caso de una paciente con un subtipo de condrosarcoma de bajo grado infrecuente en su presentación y localización, quien fue diagnosticada mediante inmunohistoquímica y tratada quirúrgicamente para lograr un salvamento exitoso de su extremidad.

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.

Estado actual del condrosarcoma en un centro de referencia / Current status of chondrosarcoma in a reference center

Resumen: Introducción: El condrosarcoma es el segundo tumor óseo maligno primario más frecuente, se caracteriza por la formación de matriz condroide, es más común en edades avanzadas y su pronóstico guarda relación directa con el subtipo histológico y el grado tumoral. El objetivo de este artículo es presentar el perfil demográfico y de tratamiento en un centro de referencia. Material y métodos: Se realizó un estudio retrospectivo, descriptivo, observacional, analítico de tipo cohorte en un centro de referencia entre el 01 de Enero de 2005 y el 31 de Diciembre de 2019, se analizaron las variables demográficas y se contrastaron con las variables de desenlace que fueron supervivencia global y período libre de enfermedad. Resultados: Se incluyeron 85 pacientes, la media edad de presentación fue 43.3 años, la etapa clínica al momento de la presentación fue mayormente IIa y IIb abarcando 54.1%, 35 pacientes tuvieron metástasis con etapa clínica IV. El principal sitio de metástasis fue el pulmón en 32 pacientes (37.6%). De los pacientes, 36 (42.3%) presentaron mortalidad cáncer específica, 35 (41.2%) fallecieron como consecuencia de la enfermedad metastásica a pulmón u otros órganos y un paciente falleció por extensión directa del tumor en columna cervical. Conclusión: Los factores pronósticos importantes hallados en nuestra serie que influyen en la supervivencia global son: la presencia de metástasis y la localización axial.

Abstract: Introduction: Chondrosarcoma is the second most frequent primary malignant bone tumor, is characterized by the formation of a chondroid matrix, is more frequent in advanced ages and its prognosis is directly related to the histological subtype and tumor grade. The objective of this article is to present the demographic and treatment profile in a reference center. Material and methods: A retrospective, descriptive, observational, analytical cohort-type study was conducted in a reference center between January 1, 2005 and December 31, 2019, the demographic variables were analyzed and contrasted with the outcome variables that were overall survival and disease-free period. Results: 85 patients were included, the mean age of presentation was 43.3 years, the clinical stage at the time of presentation was mostly IIa and IIb covering 54.1%. 35 patients had metastases with clinical stage IV. The main site of metastasis was the lung in 32 patients (37.6%). Thirty-six patients (42.3%) had specific mortality. 35 patients (41.2%) died as a result of metastatic disease to lung or other organs, one patient died from direct extension of the tumor in the cervical spine. Conclusion: The important prognostic factors found in our series that influence global survival are: the presence of metastases and axial localization.

Condrosarcoma de base de cráneo: análisis clínico-patológico de un caso

Introducción: El condrosarcoma es un tumor maligno poco común; la localización craneal es rara. En ocasiones, su resección total no es posible debido a la proximidad de la masa a estructuras vasculares y neurales importantes. Caso Clínico: Mujer de 56 años que presentó cefalea en el lado derecho, parestesia en el lado derecho del labio superior, pérdida de la memoria a corto plazo y ageusia. La RM mostró una masa extraaxial realzada. La paciente tuvo una resección quirúrgica completa. Los resultados anatomopatológicos mostraron un condrosarcoma bien diferenciado de la base del cráneo. Conclusión : El condrosarcoma es un tumor maligno con diferenciación pura del cartílago hialino, representa el 0.15 % de todos los tumores intracraneales, siendo este el segundo caso de localización de la base del cráneo reportado en el Perú. Las características histológicas más los marcadores de inmunohistoquímica revelaron características clásicas de este tumor. El tratamiento aún depende de la individualización de cada caso.

Introduction: Chondrosarcoma is an unusual malignant neoplasm; cranial location is rare. Sometimes its complete resection is not possible because of vicinity of the mass to important vascular and neural structures. Clinical case: This is a 56-year old female subject who developed headache in the right side, paresthesia in the right side of her upper lip, short term memory loss, and ageusia. A magnetic resonance imaging study showed an enhanced extra-axial mass. This patient underwent complete surgical resection. The anatomopathological study showed a well differentiated chondrosarcoma of the cranial base. Conclusion: Chondrosarcoma is a malignancy with pure differentiation of the hyaline cartilage. It represents 0.15% of all intracranial tumors, and this is the second case with a cranial base location reported in Peru. Both histological characteristics and immunohistochemistry markers revealed the classic characteristics of this tumor. Therapy is given on an individualized basis.

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.

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.