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.

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.

Primary intraocular chondrosarcoma in a dog / Condrossarcoma intraocular primário em cão

A five-year-old male Cocker Spaniel was presented for evaluation of the right eye due to discomfort, abundant purulent discharge and progressive enlargement of the eyeball. The owner revealed that the right eye has appeared to be inflamed and smaller then the left eye for years. Ophthalmic examination revealed corneal perforation, buphthalmia and conjuctival hyperemia. Enucleating was performed due to signs of endophthalmitis and ocular discomfort. Histopathology revealed a multilobulated proliferation of chondrocytes producing hyaline cartilage with occasional pleomorphism and binucleate cells. A diagnosis of primary intraocular chondrosarcoma was done.

Foi atendido um cão, da raça Cocker Spaniel, de cinco anos de idade, com desconforto ocular, secreção purulenta abundante e aumento progressivo do bulbo ocular. Ao exame oftálmico, evidenciaram-se perfuração corneana, buftalmia e hiperemia conjuntival. Foi realizada enucleação em decorrência do desconforto ocular intenso e dos sinais de endoftalmite. Exame histopatológico revelou proliferação multilobulada de condrócitos produzindo cartilagem hialina com pleomorfismo ocasional e células binucleadas. Foi diagnosticado condrossarcoma intraocular primário.

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.

Mast cells density in fibrotic capsule of enchondroma and well-differentiated chondrosarcoma: a method for histopathologic differentiation

An enchondroma is a benign and a well-differentiated chondrosarcoma is an invasive chondroid tumor with high recurrence potential. In spite of biologic differences, these two tumors have very similar histopathologic appearance. It has been shown that the biologic nature of the connective tissue around benign and malignant tumors varies in the number of mast cells. The aim of this study was to study the histopathologic distinction of enchondroma and well-differentiated chondrosarcoma using the density of the mast cells in fibrotic capsule. Twelve enchondroma and 15 well-differentiated chondrosarcoma were collected from Pathology department of Cancer Institute and Central Pathology department of Imam Khomeini Hospital in Tehran. 3 micron paraffin embedded tissue sections were stained by toluidine blue for mast cells counting. Mast cells were counted in fibrous capsule of all cases. Mast cells counts were accomplished in 10 high power fields .The average number of mast cells in 10HPF was determined as an index for each lesion. Mann-Whitney U test was used for statistical analysis. Mean index in enchondroma and well-differentiated chondrosarcoma groups were 0.1 +/- 0.12 and 0.31 +/- 0.33 respectively, showing a significant difference between number of mast cells in the fibrotic capsule in these two lesions [p=0.028]. Comparison of the corresponding points in ROC curve, showed a cut-off point = 0.15, with positive predictive value of 61%, negative predictive value 71%, specificity of 33.3% and sensitivity of 66.7%, [p=0.025]. Average density of the mast cells in the surrounding fibrotic capsules of enchondroma and well-differentiated chondrosarcoma along with other criterions, could be a beneficial factor for histologically differentiation between these two lesions

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.

Condrosarcoma de pulmón: reporte de un caso / Lung chondrosarcoma: report of a case

El condrosarcoma primitivo de pulmón es una neoplasia maligna de crecimiento lento y escasos síntomas respiratorios al inicio de la enfermedad. El objetivo de este trabajo es dar a conocer un tumor de pulmón poco frecuente, la evolución del mismo y la revisión de la literatura. El caso descrito en particular presenta disimilitudes con la literatura en cuanto a las manifestaciones clínicas, particularmente en el carácter de la tos y su causa, así como por la ausencia de hemoptisis. Por otra parte, el derrame pleural, solo se ha descrito en algunos casos de este tipo de tumor y el mismo es más frecuente en los tumores de estirpe epitelial. El condrosarcoma primario de pulmón, es una neoplasia que se diferencia por la histología y los estudios de inmunotinción, que por su historia natural amerita el diagnóstico oportuno para emplear la terapéutica que rinda los mejores beneficios y mejore la supervivencia.

The primitive lung chondrosarcoma is a malignant tumor of slow growth and low respiratory symptoms at the beginning of the course of the disease. The aim of this paper is to present a rare lung tumor, its evolution and the literature review. The case described in particular presents dissimilarities with the world literature regarding in the clinical manifestations, particularly in the character of the cough and its cause, and by the absence of the hemoptysis. Moreover, the pleural effusion, only been described in some cases of patients with this tumor type and the same is more common describe in the epithelial tumors. The primary chondrosarcoma of the lung is aneoplasm that is well differentiated by the histology and the immunohistochemistry studies, which by its the natural history warrants of the early diagnosis for the type of therapeutic uses, will render the best benefits and improvesurvival in the patients.

Analysis of angiogenic factors and cyclooxygenase-2 expression in cartilaginous tumors: clinical and histological correlation

OBJECTIVES: To study the role of angiogenesis and cyclooxygenase-2 expression in cartilaginous tumors and correlate these factors with prognosis. INTRODUCTION: For chondrosarcoma, the histological grade is the current standard for predicting tumor outcome. However, a low-grade chondrosarcoma can follow an aggressive course-as monitored by sequential imaging techniques-even when it is histologically indistinguishable from an enchondroma. Therefore, additional tools are needed to help identify the biological potential of these tumors. The degree of angiogenesis that is induced by the tumor could assist in this task. Angiogenesis can be quantified by measuring the expression of vascular endothelial growth factor and CD34, and cyclooxygenase-2 can induce angiogenesis by stimulating the production of proangiogenic factors. METHODS: In total, 21 enchondromas and 58 conventional chondrosarcomas were studied by examining the clinical and histopathological findings in conjunction with the immunostaining markers of angiogenesis and cyclooxygenase- 2 expression. RESULTS: The significant variables that were associated with poor outcome were 1) higher-grade chondrosarcomas, 2) tumors that developed in flat bones, and 3) over-expression of CD34 (with a median count that was higher than 5.9 vessels in 5 high power fields). Moreover, CD34 expression (measured using the Chalkley method) revealed significantly higher microvessel density in flat bone chondrosarcomas. DISCUSSION: Previous studies have shown a positive correlation between Chalkley microvessel density and histological grade; however, in our sample, we found that the former is predictive of the outcome. Chondrosarcomas in flat bones have been shown to correlate with a poor prognosis. We also found that CD34 microvessel density values were significantly higher in flat-bone chondrosarcomas. This could explain-at least in part-the more aggressive biological course that is taken by these tumors. CONCLUSIONS: These results provide evidence that CD34 microvessel density in chondrosarcomas can be helpful in predicting patient outcome and may add to our understanding of chondrosarcoma pathogenesis.

Alternativas quirúrgicas en el tratamiento de los tumores: de la pared torácica / Surgical alternative for the treatment of thoracic wall tumor

Los tumores de la pared torácica son neoplasias poco frecuentes, y de difícil tratamiento. Cuatro pacientes con neoplasias de la pared torácica fueron intervenidos en el Hospital Universitario de Maracaibo entre los años 2005 a 2008, con los diagnósticos de: plasmocitoma en dos pacientes y condrosarcoma en dos pacientes. Entre las intervenciones quirúrgicas realizadas destacan: resección de tumor de la pared torácica e interposición de malla en tres pacientes y resección de tumor esternal con interposición de malla y metacrilato en un paciente. La evolución posoperatoria fue satisfactoria en tres pacientes, un paciente en quien se interpuso malla y metacrilato se produjo el desplazamiento de la malla a un año de la intervención. Es importante en los pacientes con tumores de la pared torácica una adecuada planificación preoperatoria de la intervención para disminuir la morbilidad y mortalidad posoperatoria, y lograr mejores resultados quirúrgicos y funcionales.

The chest wall tumors are neoplasm with a low frequency and they are of difficult in the treatment. Four patients with chest wall neoplasm were operated in the Universitary Hospital from Maracaibo between 2005 at 2008 with the diagnoses of plasmacytoma in two patients and chondrosarcoma in two patients. The surgical procedures for us performed were: Chest wall tumor resection and mesh interposition in three patients and sternum tumor resection with interposition of mesh with methyl methacrylate in one patient. The postoperatory evolution was satisfactory in three patients and the patient with the reconstruction using mesh and methyl methacrylate had the displacement of the mesh after of a year of the surgical procedure. It is very important in the patients with chest wall tumors an adequate preoperatory evaluation with the objective of reduces the mortality and morbidity postoperatory and obtains better surgical and functional results.

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.

Chondroid lipoma in a child.

Chondroid lipoma (CL), a rare lipoma variant, has not been described well for patients younger than 14 years of age. We herein report an extremely unusual instance of CL in a 7-year-old child. The cut surface of the tumor showed peculiar tan-brown and yellow-white areas. Histopathological sections showed the presence of eosinophilic variably vacuolated round- to oval-spindle cells in a myxo-hyaline background. The findings were consistent with CL. The case is reported because of its extreme rarity.

Matrix-producing mammary carcinoma: a rare breast tumor.

Matrix-producing carcinoma of the breast is a unique subclass of metaplastic carcinoma which is characterized by the existence of a ductal carcinomatous component with direct transition to areas showing cartilagenous or osseous differentiation, lacking an interspersed spindle cell component. This article reports one such rare case in a 50-year-old woman who had a right breast mass. f0 ine needle aspiration (FNA) smears showed abundant chondromyxoid extracellular matrix to which were variably admixed carcinomatous cells. Histological examination revealed a neoplasm composed of invave ductal carcinoma with a direct transition to chondrosarcomatous areas. The case is reported not only for its peculiar microscopic characteristics but also to highlight its better prognostic features and hence, the need for its recognition.

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.

Malignant mixed tumor in the salivary gland of a cat

The presence of a malignant mixed tumor, also known as a carcinosarcoma, in the salivary gland is very rare. Such mors, which are typically aggressive, are characterized by the presence of carcinomatous and sarcomatous components. 9-year-old neutered female domestic short-haired cat presented with swelling in the right mandibular lesion that had rapidly enlarged over the previous 3 weeks. Physical examination revealed a large, fluctuated and painless subcutaneous swelling that was associated with a firm mass. Radiographs of the head revealed a soft-tissue density that involved faint circular calcific opacity. Contrast-enhanced computed tomography revealed that the peripheral capsulated cystic area had a contrast enhanced region without bone lysis. The cat received a total excision of the mass and postoperative radiotherapy. Histopathological analysis of the mass revealed that it was a malignant mixed tumor. Metastasis to the lung was discovered 7 weeks later, at which time treatment was stopped.

Primary chondrosarcoma in the skull of a dog

Chondrosarcoma of the skull is a rare primary malignant tumor that is slow-growing, but locally aggressive. A 5-year-old, golden retriever was presented to our hospital with a swelling in the left side of her head, and the swelling had slowly enlarged over the previous month. There were no significant changes on the neurological examination. A computed tomography scan revealed a large mass involving bone destruction and prominent matrix mineralization. T1-weighted magnetic resonance imaging showed a slightly low-signal intensity area and a T2-weighted image revealed marked, high-signal intensity. There was compression of the adjacent brain parenchyma. Histopathological examination confirmed the lesion to be a chondrosarcoma.