Mesenchymal chondrosarcoma - A rare cause of pleural effusion.

Mesenchymal chondrosarcoma is a malignant neoplasm arising from cartilaginous bone or soft tissue. It is uncommon yet devastating. Our patient was a 21-year-old man who presented with pleuritic chest pain and weight loss. His chest radiograph showed left pleural effusion. His pleural effusion analysis was consistent with exudative pleural effusion. Tuberculosis workup was negative. Pleural fluid cytology did not yield malignant cells. Subsequently, his computed tomography of thorax showed left rib sclerotic lesion with soft tissue component. Biopsy of the soft tissue eventually confirmed the diagnosis of mesenchymal chondrosarcoma. He succumbed to his illness before the diagnosis was confirmed. We hope that through this case report, we are able to provide some insight into this rare condition.

Mesenchymal chondrosarcoma metastasising to the pancreas.

The mesenchymal chondrosarcoma (MC) is a rare malignant tumour and accounts for less than 3% of primary chondrosarcomas. Mostly MC arises from the craniofacial bones, the ribs, the ilium, the femur and the vertebrae. A 54-year-old man was treated due to an icterus of unknown origin. The medical history of the patient consists of a multimodal treated MC of the thoracic vertebrae. A CT imaging identified a 2×4 cm sized mass of the pancreatic head. Suspecting a pancreatic head carcinoma surgical removal was performed. Histopathological a metastasis of MC was diagnosed. Our patient left the hospital after 17 days and died 23 month after surgery. Metastases of MC to the pancreas are rare. When detecting a mass of the pancreas in patients with a medical history of an MC, a metastasis of these tumour should be taken in consideration.

Spontaneous Intraventricular Hemorrhage: A Rare Presentation of a Skull Base Mesenchymal Chondrosarcoma.

BACKGROUND: Chondrosarcomas are very rare malignant, slow-growing tumors that develop in or near the petroclival region of the brain. We report a very rare case in which the tumor originated from left petrous bone and induced intraventricular hemorrhage leading to an acute comatose presentation. CASE DESCRIPTION: A 28-year-old man initially presented to the outpatient department with a 1-month history of headache, vomiting, vertigo, and left facial numbness. A lesion at the cerebellopontine angle with extension into the middle cranial fossa was demonstrated on computed tomography and magnetic resonance imaging. The following night his condition worsened, and he presented to the emergency department with intraventricular hemorrhage with hydrocephalus. An external ventricular drain was placed in the emergency department to relieve hydrocephalus, and definitive surgical resection of the tumor was subsequently. Postoperatively, his Glasgow Coma Scale score improved, and he was transferred to the surgical intensive care unit where he remained for 3 days. He was subsequently stepped down to a special care unit and then to a ward room. The patient is currently awake and has grade II facial palsy (House-Brackmann), demonstrates spontaneous purposeful eye opening, inconsistently obeys single-step orders, demonstrates no meaningful phonation or vocalization, and has at least grade 4 power in all 4 extremities. He is currently fed through a nasogastric tube and is in rehabilitation. CONCLUSIONS: Our experience of petroclival junction chondrosarcoma causing intraventricular hemorrhage may be the first to be documented. Preferred treatment of this highly malignant lesion is radical removal with postoperative radiotherapy.

Schwannoma gástrico como hallazgo ecográfico casual / Gastric schwannoma presenting as a casual ultrasonographic findings

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Primary intraspinal dumbbell-shaped mesenchymal chondrosarcoma with massive calcifications: a case report and review of the literature.

BACKGROUND: Mesenchymal chondrosarcoma is a rare malignant tumor arising from bone or soft tissues. Instraspinal dumbbell-shaped mesenchymal chondrosarcoma is even rarer; however, it should not be neglected by clinicians. CASE PRESENTATION: A 26-year-old female was referred to our hospital with a 1.5-month history of sciatic pain and numbness in the left leg. Computed tomography and magnetic resonance imaging scans revealed an intraspinal dumbbell-shaped mass which had distinguishing features of neurogenic tumors, surprisingly, with massive calcifications, and no tumor metastasis was found. Then the patient underwent a total resection of the tumor, and during the operation, we found that the right nerve root of the fifth lumbar almost disappeared. The tumor was diagnosed as mesenchymal chondrosarcoma by histopathological examination after operation. Adjuvant therapies were not performed. However, recurrence of the tumor occurred 5 months later, and she underwent a total resection again combined with radiotherapy after second surgery. CONCLUSIONS: To the best of our knowledge, this case study presents the first report in literature about primary instraspinal dumbbell-shaped mesenchymal chondrosarcoma with massive calcifications, which may provide some evidence for clinical practice. As the clinical symptoms and radiographic findings of mesenchymal chondrosarcoma are usually not specific, clinicians should consider it as a possible case and diagnose it through careful histopathological examination. Sometimes, calcification could be seen in tumors, which may influence or reflect the growth of tumor and disease prognosis. Although prognosis in mesenchymal chondrosarcoma varies from person to person, generally, complete resection, adjuvant therapy, and regular examinations are recommended to perform for patients with mesenchymal chondrosarcoma.

Tumores mesenquimales de intestino delgado: descripción de dos casos inusuales / Small bowel mesenchymal tumors: description of two unusual cases

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Angiomixoma agresivo. Un raro tumor mesenquimal pélvico / Aggressive angiomyxoma. A rare mesenchymal pelvic tumor

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Osteomalacia en un adulto joven / Osteomalacia in a young adult

Los cuadros de osteomalacia hipofosfatémica responden a diversas causas genéticas y adquiridas. Algunas variantes de tumores mesenquimales producen cantidades inapropiadas de factor de crecimiento fibroblástico 23 (FGF-23), un mediador que induce una pérdida renal de fosfatos. El cuadro bioquímico se caracteriza por hipofosfatemia, disminución de la reabsorción tubular de fosfatos, niveles bajos o inapropiadamente normales de calcitriol sérico y niveles altos o inapropiadamente normales de FGF-23 plasmático. Este síndrome paraneoplásico es denominado osteomalacia tumoral u oncogénica. Existen limitadas series de casos publicadas, pero su reconocimiento es creciente en los últimos años. El diagnóstico puede ser complejo por su baja incidencia, la dificultosa localización de los tumores y la heterogeneidad en la interpretación histopatológica. La exéresis quirúrgica completa es curativa, pero puede haber recidivas y los suplementos orales de fósforo y calcitriol son alternativas de tratamiento médico (AU)

Cases of hypophosphatemic osteomalacia respond to various causes, both genetic and acquired. Some variants of mesenchymal tumors produce inappropriate amounts of fibroblast growth factor 23 (FGF-23), a mediator which induces renal phosphate loss. The biochemical picture is characterized by hypophosphatemia, decreased tubular reabsorption of phosphates, low or inappropriately normal serum calcitriol and high or unusually normal levels of FGF-23 plasma. This paraneoplastic syndrome is called tumorinduced or oncogenic osteomalacia. There are a limited series of published cases, although it has been increasingly accepted in recent years. Diagnosis may be complex given its low incidence, the difficulties in localizing the tumors and heterogeneity in histopathologic interpretation. Complete surgical removal has healed, but there may be recurrences whereas phosphorus and calcitriol oral supplements offer alternative medical treatment (AU)

Pulsed radiofrequency treatment of piriformis syndrome in a pregnant patient with malignant mesenchymal tumor.

Cancer is frequently seen in women of reproductive age. Diagnosis, management of treatment, and safety of the therapeutic approach are particularly important for these patients. Presently described is pain management in a case of pregnancy with malignant mesenchymal tumor. A 23-year-old woman in 30th gestational week presented with severe pain in right hip and back of the right thigh. Piriformis block successfully decreased pain and was followed by pulsed radiofrequency (PRF) to the piriformis muscle. PRF, as a non-neurodestructive method, is a safe and effective method to treat cancer pain in a pregnant patient.

Cervical subtotal en-bloc spondylectomy of C6 mesenchymal chondrosarcoma.

INTRODUCTION: We present a case of C6 mesenchymal chondrosarcoma and discuss safe posterior to anterior approach subtotal en-bloc spondylectomy. MATERIALS AND METHODS: A 29-year-old male consulted for our department with severe posterior neck pain doing exercise. CT scan demonstrated a primary osteolytic lesion on C6 left transverse foramen and MRI demonstrated the tumor involved C6 vertebra from layers B, C and F sectors 4-6 encasing left vertebral artery. Preoperatively neurointerventional radiology service occluded the left vertebral artery and tumor feeding artery using coil embolization. Posterior approach consist of C5-C7 laminectomy, left sided C6 and C7 nerve root sacrifice, posterior disc removal and release of C5-6-7 and posterior reconstruction. Then, position was changed to supine, and the anterior approach was followed as C5-6, C6-7 discectomy, left vertebral artery ligation and cut, longus coli resection and C6 subtotal spondylectomy with en-bloc resection of mass, mesh cage insertion and C5-C7 anterior plate fixation. During operation, frozen biopsy was performed on 8 areas (longus coli, lateral margin, anteroinferior margin, posterior margin, posterosuperior margin, C5 transverse foramen, posteroinferior margin, inferior margin) after wide resection. Tumor free margin was confirmed. RESULTS: After operation, he complained of tingling sensation of left thumb and forearm medial side, and elbow extensor motor grade was checked to 4/5 postoperatively. In the followed-up radiograph, the tumor was completely removed, and the instability of joint was not seen. As a result of observing follow-up CT at a year after the surgery, recurrence findings have not been shown up to now, and the progression of neurologic symptoms has not been shown either. CONCLUSION: Based on the Grand Round case and relevant literature, we discuss the case of mesenchymal chondrosarcoma occurring from the C6 cervical spine treated with cervical subtotal en-bloc spondylectomy. Successful en-bloc resection of the tumor was achieved using posterior to anterior approach.

Tumor fibrohistiocítico plexiforme con metástasis ganglionar: reporte de un caso / Plexiform fibrohistiocytic tumor with nodal metastasis: a case report

El tumor fibrohistiocítico plexiforme (TFHP) es una rara neoplasia mesenquimal de malignidad intermedia, con alta tasa de recidiva local y potencial metastásico ganglionar o pulmonar. Suele afectar tejidos blandos superficiales de extremidades superiores de niños y adultos jóvenes, con predilección femenina. Presentamos el caso de un niño de 10 años con tumoración de crecimiento progresivo, dependiente del plano cutáneo en rodilla derecha con adenopatías inguinales ipsilaterales. Se le realiza resección amplia de la lesión con linfadenectomía inguinocrural. El estudio histopatológico reveló una neoplasia de patrón plexiforme con componente bifásico (células mononucleares tipo histiocitos asociado a células gigantes tipo osteoclasto, CD68 positivas y células fusiformes, actina de músculo liso positivas); favoreciendo el diagnóstico de esta entidad con metástasis ganglionar (AU)

Plexiform fibrohistiocytic tumour (TFHP) is a rare mesenchymal neoplasm of intermediate malignancy but with a high rate of local recurrence and potential for lymph node or pulmonary metastases. It usually affects superficial soft tissues of the upper extremities of children and young adults and is more frequent in females. We report a case of a 10 year old boy with progressive tumour growth, under the skin in the right knee with ipsilateral inguinal lymphadenopathy. He underwent wide resection of the lesion with inguinal lymphadenectomy. Histopathology revealed a plexiform pattern with a biphasic component (mononuclear histiocytic cells associated with CD68 positive osteoclast-like giant cells and smooth muscle actin-positive spindle cells); favoring the diagnosis of TFHP with lymph node metastasis (AU)

Potencial terapéutico de las células madre derivadas de la médula ósea en el infarto cerebral / Therapeutic potential of bone marrow stem cells in cerebral infarction

Introducción. Las células madre constituyen una alternativa terapéutica que se encuentra en fase de experimentación para el infarto cerebral. Objetivo. Mostrar la evidencia científica existente sobre el potencial terapéutico de las células madre de la médula ósea en esta enfermedad. Desarrollo. El infarto cerebral representa el 80% de las enfermedades cerebrovasculares. La trombólisis constituye la única terapia aprobada, pero, por su estrecha ventana terapéutica, sólo se aplica a un bajo porcentaje de los pacientes. De manera alternativa, los tratamientos neurorrestauradores, como el de células madre, pueden aplicarse en períodos más prolongados. Por esta razón se efectuó una búsqueda bibliográfica en PubMed con el empleo de las palabras clave ‘stem cells’, ‘bone marrow derived mononuclear cells’ y ‘stroke’. Se encontraron evidencias de seguridad y eficacia de dichas cé- lulas en diferentes momentos evolutivos del infarto cerebral. Se identificaron estudios que en clínica y preclínica las recolectaron por punción medular y en sangre periférica, y las trasplantaron directamente en el área infartada o por vía intravascular. El efecto terapéutico se relaciona con sus propiedades de plasticidad celular y liberación de factores tróficos. Conclusiones. El concentrado de células mononucleares autólogas, obtenido en sangre periférica o por punción de la médula ósea, y trasplantado por vía intravenosa, es una factible opción metodológica que permitirá rápidamente incrementar el número de ensayos clínicos en diferentes etapas evolutivas del infarto cerebral. Esta terapia muestra seguridad y eficacia; sin embargo, deben ampliarse las evidencias que avalen su generalización en humanos (AU)

Introduction. Stem cells are an alternative therapy for cerebral infarction that is still in the experimental phase. Aims. To report on the existing scientific evidence on the therapeutic potential of bone marrow stem cells in this disease. Development. Cerebral infarction accounts for 80% of cerebrovascular diseases. Thrombolysis is the only approved therapy, but, owing to its narrow therapeutic window, it is only applied to a low percentage of patients. Conversely, neurorestorative treatments, such as stem cells, can be applied over longer periods of time. For this reason a literature search was conducted on PubMed using the key words ‘stem cells’, ‘bone marrow derived mononuclear cells’ and ‘stroke’. Evidence was found of the safety and effectiveness of such cells at different points in the development of the completed stroke. Results included studies that, in the clinical and preclinical period, collected them by spinal puncture and in peripheral blood, and transplanted them either directly into the infarcted area or intravenously. The therapeutic effect is related with their cell plasticity and trophic-factor releasing properties. Conclusions. Autologous mononuclear cell concentrate, obtained from peripheral blood or by puncturing the bone marrow and transplanted intravenously, is a feasible methodological option that will make it possible to quickly increase the number of clinical trials conducted at different stages of the development of a completed stroke. This therapy has proved itself to be safe and effective; nevertheless, further evidence is needed to endorse its generalised use in humans (AU)

Angiomixoma agresivo pélvico: diagnóstico en un examen de salud periódico de una trabajadora expuesta a radiaciones ionizantes / Agressive angiomyxom of the pelvis: diagnosis in an annual health examination of a worker exposed to ionizing radiation

El angiomixoma agresivo (AA) fue descrito por Steeper y Rosai en 1983 para definir una neoplasia mesenquimal mixoide de crecimiento lento localizada en la región pélvica, genital y/o perineal. Se tratan de tumores benignos, poco frecuentes, infiltrativos localmente, raramente metastatizantes y muy recidivantes. La prevalencia mujer/hombre es de 6:1, siendo más frecuente en la edad fértil por estar relacionada con los niveles hormonales de estrógenos y progesterona. Clínicamente el AA cursa con sintomatología inespecífica y variable en relación al tamaño tumoral y a su crecimiento lento, de ahí la importancia de realizar un diagnóstico diferencial precoz tanto macroscópico como microscópico. El diagnóstico se basa en pruebas de imagen (Resonancia Magnética, Tomografía Computarizada), histológicas e inmunohistoquímicas, siendo estas determinantes a la hora de la exéresis completa tumoral, tratamiento de elección en estos casos, al no haber sido demostrada la eficacia de otras terapias como la radio o quimioterapia

Aggressive angiomyxoma (AA) was described in 1983 by Steeper y Rosai as a myxoid mesenchymal neoplasm of the pelvic, genital and/or perineal region with slow growth. The AA is an uncommon and benign neoplasm, although it is a locally aggressive neoplasm. It is rarely metastasize and shows a high rate of recurrence. The prevalence rate female/male is 6:1, with a higher frequency in the childbearing age due to its relation with oestrogen and progesterone levels. The clinical symptoms of the AA are several and non-specific. Those symptoms are related to the size and the slow growth of the tumours. For this reason it is extremely important to do an early macroscopic and microscopic differential diagnosis. The specific diagnosis of this tumour is based on imaging test (CTscan, MRI), as well as an histopathological and immunohistochemical study, which are critical to carry out a complete surgical resection of the tumour, which is the main treatment in this case. There are no evidences of the successful of radiotherapy and chemotherapy

Hemangiomas y hamartomas hepáticos en un lactante con síndrome de Beckwith-Wiedemann asociado a displasia mesenquimal placentaria / Hepatic hemangiomas and hamartomas in children with Beckwith-Wiedemann syndrome associated with placental mesenchymal dysplasia

La displasia mesenquimal placentaria (DMP) es una rara patología que afecta al desarrollo de la vascularización placentaria y condiciona la aparición en el recién nacido de hamartomas hepáticos y alteraciones hematológicas, asociándose además en 1 de cada 4 casos al desarrollo del síndrome de Beckwith-Wiedemann (BWS), con la que comparte un origen genético común. Presentamos el caso de un recién nacido afectado de BWS asociado a DMP, que además de los hamartomas hepáticos descritos en la bibliografía, presentó como hallazgo casual lesiones hepáticas de tipo sólido con diagnóstico anatomopatológico de hemangiomas hepáticos con marcador Glut-1 positivo, molécula con implicaciones en la respuesta terapéutica y el pronóstico a largo plazo de estas lesiones. El tratamiento con propranolol es efectivo en estos casos, ya que consigue disminuir el tamaño de las lesiones, como en el caso que presentamos (AU)

The placental mesenchymal dysplasia (DMP) is a rare disease that affects the development of placental vascularization and conditions the appearance of liver hamartomas and blood disorders in newborns. In addition it is associated with Beckwith-Wiedemann syndrome (BWS) in 25% of the cases, sharing a common genetic origin. We report a case of both entities (DMP and BWS) in a male newborn, who developed not only liver hamartomas as described in the literature but also liver hemangiomas with positive marker Glut-1 as a new finding; this molecule is related to a better therapeutic response and long-term prognosis. The patient recieved treatment with propranolol with successfull reduction of the lesions size (AU)

Condrosarcoma mesenquimal del maxilar superior: un tumor raro de la cavidad oral / Mesenchymal chondrosarcoma of upper maxilla: a rare tumor of the oral cavity

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Histiocitoma fibroso maligno. Un sarcoma excepcional en la glándula mamaria / Malignant fibrous histiocytoma. An exceptional sarcoma in the mammary gland

Introducción. El histiocitoma fibroso maligno constituye la neoplasia sarcomatosa más frecuente en los adultos, pero la mama es una localización excepcional. Presentamos el caso de una mujer que comenzó con una tumoración de crecimiento progresivo en la mama derecha. Caso clínico. Mujer de 68 años que consulta por autopalpación de un nódulo en la mama derecha que ha crecido de forma progresiva en los últimos meses. La mamografía y la ecografía muestran una imagen nodular con bordes bien definidos, situada en intercuadrantes superiores de mama derecha, sin adenopatías axilares. Se decidió intervención quirúrgica y el estudio histológico definitivo fue informado como neoformación mesenquimal fusocelular con patrón estoriforme. El estudio inmunohistoquímico fue compatible con un histiocitoma fibroso maligno. Conclusión. Es primordial el diagnóstico diferencial de esta entidad clínica debido a la variabilidad histológica de los tumores sarcomatosos. Sus características clínicas y radiológicas pueden hacerlo pasar desapercibido, pero su comportamiento agresivo hace necesario un diagnóstico precoz, lo cual permitirá un tratamiento adecuado para lograr el aumento en la supervivencia(AU)

Introduction. Malignant fibrous histiocytoma is the most common sarcomatous neoplasm in adults. Localization in the breast, however, is exceptional. We report the case of a woman who presented with progressive tumoral growth in the right breast. Case report. A 68-year-old woman consulted for a self-palpated nodule in the right breast that had grown steadily in the last few months. Mammography and ultrasound showed a nodule with well-defined borders, located in the upper inner quadrant of the right breast. There was no axillary lymphadenopathy. Surgery was performed and the histological examination gave a definitive diagnosis of mesenchymal spindle cell neoplasm with storiform pattern. Immunohistochemical analysis was compatible with a diagnosis of malignant fibrous histiocytoma. Conclusion. Due to the histological variability of sarcomatous tumors, differential diagnosis is paramount in malignant fibrous histiocytoma. Because of their clinical and radiological features, malignant fibrous histiocytoma can be overlooked. Because these tumors are aggressive, an early diagnosis is essential to allow appropriate treatment and to increase survival(AU)