Pseudomyogenic hemangioendothelioma with bone and soft tissue involvement with favorable response to pamidronate: a case report and systematic review of the literature.

Pseudomyogenic hemangioendothelioma (PMH) can be a challenge for diagnosis and might be confused with other tumors, such as epithelioid sarcoma. Here we present a case and a systematic review of the literature to identify and discuss PMH treatment in primary bone involvement. A 25-year-old woman was referred for bone pain (10/10) in the left lower limb. Magnetic resonance imaging (MRI) showed multiple bone lesions (left femur, tibia, patella, ankle, and foot) with well-defined borders without signs of local aggressiveness. Positron Emission Tomography-Computed Tomography (PET-CT) showed multiple metabolic musculoskeletal lesions in the left lower limb. A CT scan-guided biopsy was performed. Histological and immunohistochemical findings confirmed the diagnosis of PMH. After treatment with intravenous pamidronate (90 mg/monthly), the patient had clinical improvement, mild pain 2/10 without the use of non-steroidal anti-inflammatory drugs or opiates. Follow-up was assessed by MRI and PET-CT. PET-CT showed metabolic resolution of most of the bone and muscular lesions and a significant improvement of the femoral lesion. MRI showed that the lesions in the left femur, tibia, and foot had a marked decrease in size without intravenous post-contrast enhancement and smaller lesions had disappeared. After a 3-year follow-up, PET-CT showed no metabolically active images. Literature review identified 31 records including 58 clinical cases of PMH with primary bone involvement and treatment description for qualitative analysis. Most lesions (69%) were treated by local excision or curettage. In addition, amputations were performed in a significant percentage of cases (20.7%). In the last years, mTOR inhibitors (n = 7) and anti-resorptive treatments (n = 4) were considered as alternative treatment options, especially in multifocal lesions.

Soft Tissue Sarcomas: Is Pre-operative Radiotherapy Associated With More Acute Wound Complications?

INTRODUCTION: Increased wound complication rates are attributed to the use of pre-operative radiotherapy. The purpose of this study is to evaluate the incidence of complications with or without pre-operative radiotherapy in our institution. METHODS: We retrospectively evaluated 48 adult patients with high-grade extremity soft tissue sarcoma. Twenty-two patients received pre-operative radiotherapy (group A) while 26 patients underwent initial surgery (group B). Complications were defined as major wound complications if they were severe enough to delay the delivery of adjuvant treatment (chemotherapy or radiotherapy) more than eight weeks after surgery or if their resolution required a new surgical intervention. RESULTS: Mean follow-up in group A and group B was 44.3 and 53.8 months, respectively. The incidence of complications of any grade in group A was 45.5% and 53.8% in group B (p: 0.566). Major wound complications in group A and group B occurred in 18% and 23% of the patients (p=0.630), respectively. All patients in group A completed local treatment - radiotherapy and surgery - in 66 days on average. In contrast, in group B post-operative radiotherapy was either delayed or suspended in four patients due to wound complications. This determined that 15.4% of the patients in group B did not receive the local treatment - surgery + radiotherapy - as planned. CONCLUSIONS: An increased risk of severe acute wound complications with the administration of pre-operative radiotherapy was not observed in patients with soft tissue sarcomas of the extremities. In addition, local treatment completion was not jeopardized with preoperative radiotherapy, as opposed to post-operative radiotherapy.

Two cases of Gorham-Stout disease with good response to zoledronic acid treatment.

Gorham-Stout disease - also called vanishing bone syndrome - is a rare bone disease characterized by a progressive intra-osseous proliferation of non-neoplastic vascular tissue resulting in massive osteolysis. Here, we report two clinical cases of Gorham-Stout disease. Case 1: a 56-year-old woman with 20 years of history of pain and swell in elbows, ankles and wrist. Then she was diagnosed as systemic lupus erythematosus (SLE) with glomerulonephritis type III. After other pathologies were ruled out Gorham-Stout disease was diagnosed. Intravenous zoledronic acid (5 mg) was indicated and after third infusion a progressive improvement of pain, mobility and daily activities were observed. Case 2: a 70-years-old man with a history of pain and limited motion in the left shoulder without X-ray abnormality. Six months later pathological fracture in the left humerus occurred and after ruled out other pathologies Gorham-Stout disease was diagnosed. Intravenous zoledronic acid (5 mg) was indicated and a good response was observed after the first infusion. Nowadays just over 200 cases were reported. Gorham-Stout disease was reported in different bones, at different age presentation and severe physical deformities, disabilities, and life-threatening complications can occur. Two cases of Gorham-Stout disease with good response to zoledronic acid was reported in this article.

Living donor bone banking: processing and discarding--from procurement to therapeutic use.

Skeletal muscle and osteoarticular tissue banks are responsible to procure, process, store and distribute tissues, from living and cadaveric donors. The procedures involve the application of protocols covering all aspects of the banking, ensuring the best tissue quality and maximum safety for the recipient. An analysis on the causes of bone tissue discarded by Biotar Tissue Bank between January 2005 and December 2012 was carried. Bone tissue was obtained from both hip and knee replacement (femoral heads and tibial plateau respectively) in living donors treated at different medical-surgical institutions in Argentina. These tissues were processed at the Bank to produce both frozen and lyophilized cancellous bone. Out of 3413 donated bones received by the Bank, 77.55 % resulted in final product, while the remaining 22.44 % was discarded in compliance with the quality standards of both the Bank and the regulatory authority. Comparing the last and the first year of the studied period, the number of discarded tissue increased 3.6 times, while the number of collected bones was approximately 10 times higher. Related to total disposed tissue, reactive serology was the most frequent cause (62.14 %), followed by inappropriate collection/storage of blood sample (30.81 %). A progressive reduction in the percentages of total discard was observed, and this was proportional to inappropriate collection/storage of blood sample. No significant differences were found in the discard rates due to positive serology throughout all the years studied. The success of a tissue bank requires full commitment of all the personnel especially the team members responsible for donor selection and the processing of allografts. It is important to critically screen donors in the early stages of donor recruitment. All of the procedures carried out by the tissue bank are parts of the quality control system which must be strictly carried out. Biotar Tissue Bank is continuously committed to ensure safety to the recipients.

Osteomalacia por tumor secretor de FGF-23 / Ostemalacia due to a tumor secreting FGF-23

Se presenta un caso de osteomalacia oncogénica en un varón de 50 años, con fuertes dolores óseos y gran debilidad muscular durante 4 años. Tenía varias deformidades vertebrales dorsales en cuña, fracturas en ambas ramas iliopubianas y en una rama isquiopubiana, y una zona de Looser en la meseta tibial derecha. Se localizó un tumor de 2 cm de diámetro en el hueco poplíteo derecho mediante centellograma con octreótido marcado con tecnecio. El tumor fue extirpado quirúrgicamente. La microscopía mostró un tumor mesenquimático fosfatúrico, de tejido conectivo mixto. La inmunotinción demostró FGF-23. Hubo rápida mejoría, con consolidación de las fracturas pelvianas y de la pseudofractura tibial y normalización de las alteraciones bioquímicas.(AU)

A case of oncogenic osteomalacia in a 50-year-old male is here presented. He suffered severe bone pain and marked muscular weakness of 4 years duration. There were several vertebral deformities in the thoracic spine, bilateral fractures of the iliopubic branches, another fracture in the left ischiopubic branch, and a Loosers zone in the right proximal tibia. An octreotide-Tc scan allowed to identify a small tumor in the posterior aspect of the right knee. It was surgically removed. Microscopically, it was a phosphaturic mesenchymal tumor-mixed connective tissue (PMT-MCT). Expression of FGF-23 was documented by immune-peroxidase staining. There was rapid improvement, with consolidation of the pelvic fractures and the tibial pseudo-fracture. The laboratory values returned to normal.(AU)

Osteomalacia por tumor secretor de FGF-23 / Ostemalacia due to a tumor secreting FGF-23

Se presenta un caso de osteomalacia oncogénica en un varón de 50 años, con fuertes dolores óseos y gran debilidad muscular durante 4 años. Tenía varias deformidades vertebrales dorsales en cuña, fracturas en ambas ramas iliopubianas y en una rama isquiopubiana, y una zona de Looser en la meseta tibial derecha. Se localizó un tumor de 2 cm de diámetro en el hueco poplíteo derecho mediante centellograma con octreótido marcado con tecnecio. El tumor fue extirpado quirúrgicamente. La microscopía mostró un tumor mesenquimático fosfatúrico, de tejido conectivo mixto. La inmunotinción demostró FGF-23. Hubo rápida mejoría, con consolidación de las fracturas pelvianas y de la pseudofractura tibial y normalización de las alteraciones bioquímicas.

A case of oncogenic osteomalacia in a 50-year-old male is here presented. He suffered severe bone pain and marked muscular weakness of 4 years' duration. There were several vertebral deformities in the thoracic spine, bilateral fractures of the iliopubic branches, another fracture in the left ischiopubic branch, and a Looser's zone in the right proximal tibia. An octreotide-Tc scan allowed to identify a small tumor in the posterior aspect of the right knee. It was surgically removed. Microscopically, it was a phosphaturic mesenchymal tumor-mixed connective tissue (PMT-MCT). Expression of FGF-23 was documented by immune-peroxidase staining. There was rapid improvement, with consolidation of the pelvic fractures and the tibial pseudo-fracture. The laboratory values returned to normal.

[Ostemalacia due to a tumor secreting FGF-23]. / Osteomalacia por tumor secretor de FGF-23.

A case of oncogenic osteomalacia in a 50-year-old male is here presented. He suffered severe bone pain and marked muscular weakness of 4 years' duration. There were several vertebral deformities in the thoracic spine, bilateral fractures of the iliopubic branches, another fracture in the left ischiopubic branch, and a Looser's zone in the right proximal tibia. An octreotide-Tc scan allowed to identify a small tumor in the posterior aspect of the right knee. It was surgically removed. Microscopically, it was a phosphaturic mesenchymal tumor-mixed connective tissue (PMT-MCT). Expression of FGF-23 was documented by immune-peroxidase staining. There was rapid improvement, with consolidation of the pelvic fractures and the tibial pseudo-fracture. The laboratory values returned to normal.

Osteomalacia por tumor secretor de FGF-23. / [Ostemalacia due to a tumor secreting FGF-23].

A case of oncogenic osteomalacia in a 50-year-old male is here presented. He suffered severe bone pain and marked muscular weakness of 4 years duration. There were several vertebral deformities in the thoracic spine, bilateral fractures of the iliopubic branches, another fracture in the left ischiopubic branch, and a Loosers zone in the right proximal tibia. An octreotide-Tc scan allowed to identify a small tumor in the posterior aspect of the right knee. It was surgically removed. Microscopically, it was a phosphaturic mesenchymal tumor-mixed connective tissue (PMT-MCT). Expression of FGF-23 was documented by immune-peroxidase staining. There was rapid improvement, with consolidation of the pelvic fractures and the tibial pseudo-fracture. The laboratory values returned to normal.

Cultivo de condrocitos sobre una matriz acelular derivada de membrana amniocoriónica / Culture of chondrocytes on an acellular matrix derived from amniochorionic membrane

Introducción: El tejido cartilaginoso articular presenta escasa capacidad regenerativa. Existe alta incidencia de lesiones condrales en la rodilla, especialmente de grado II/III (Outerbridge). El uso combinado de células autólogas cultivadas con membranas biológicas es una posibilidad terapéutica. El objetivo del presente trabajo es analizar las características del desarrollo in vitro de condrocitos humanos sobre una membrana amniocoriónica acelular (MAC) desecada. Materiales y métodos: Entre diciembre de 2010 y diciembre de 2011 se procesaron 16 muestras de cartílago de donante vivo, de las cuales se analizaron siete. Los condrocitos fueron cultivados y amplificados sobre plástico, a partir de lo cual se realizaron los siguientes análisis: interacción entre células y MAC, capacidad de la MAC como matriz para las células y comportamiento de las células cultivadas sobre la MAC. Resultados: Los condrocitos in vitro mostraron cambios fenotípicos en presencia de MAC. Las células fueron capaces de adherirse y permanecer en la región esponjosa de la membrana. La microscopia electrónica de las MAC cultivadas mostró la presencia de células, organelas celulares bien conservadas, retículo endoplásmico y uniones de tipo desmosoma. Conclusiones: Este trabajo muestra la factibilidad de cultivar condrocitos sobre MAC. Las células fueron capaces de adherirse, permanecer y diferenciarse sobre la membrana durante el tiempo del estudio (AU)

Cultivo de condrocitos sobre una matriz acelular derivada de membrana amniocoriónica / Culture of chondrocytes on an acellular matrix derived from amniochorionic membrane

Introducción: El tejido cartilaginoso articular presenta escasa capacidad regenerativa. Existe alta incidencia de lesiones condrales en la rodilla, especialmente de grado II/III (Outerbridge). El uso combinado de células autólogas cultivadas con membranas biológicas es una posibilidad terapéutica. El objetivo del presente trabajo es analizar las características del desarrollo in vitro de condrocitos humanos sobre una membrana amniocoriónica acelular (MAC) desecada. Materiales y métodos: Entre diciembre de 2010 y diciembre de 2011 se procesaron 16 muestras de cartílago de donante vivo, de las cuales se analizaron siete. Los condrocitos fueron cultivados y amplificados sobre plástico, a partir de lo cual se realizaron los siguientes análisis: interacción entre células y MAC, capacidad de la MAC como matriz para las células y comportamiento de las células cultivadas sobre la MAC. Resultados: Los condrocitos in vitro mostraron cambios fenotípicos en presencia de MAC. Las células fueron capaces de adherirse y permanecer en la región esponjosa de la membrana. La microscopia electrónica de las MAC cultivadas mostró la presencia de células, organelas celulares bien conservadas, retículo endoplásmico y uniones de tipo desmosoma. Conclusiones: Este trabajo muestra la factibilidad de cultivar condrocitos sobre MAC. Las células fueron capaces de adherirse, permanecer y diferenciarse sobre la membrana durante el tiempo del estudio