Plexiform fibromyxoma of the stomach: An underrecognized entity!

Plexiform fibromyxoma (PF) is a recently described rare type of mesenchymal tumor of the stomach with only 123 cases reported in the literature. It is characterized by a peculiar plexiform growth pattern, myxoid stroma with arborizing microvasculature, and spindle-shaped myofibroblastic cells. We herein report a case of gastric PF in a 15-year-old boy, mimicking a gastrointestinal stromal tumor (GIST) due to overlapping clinicoradiological features. Distinct pathological and immunohistochemical features of PF do aid in distinction from GIST and other mesenchymal entities. Diagnosis is crucial as surgical resection is the mainstay of treatment unlike aggressive management in GIST. It is a benign entity with no local recurrence or distant metastasis reported so far, but confirmation of the same requires longitudinal observational studies with a larger sample size.

Hemangiopericitoma en el cuello / Hemangiopericytoma in the Neck

Introducción: El hemangiopericitoma es un raro tumor mesenquimal (vascularizado y potencialmente maligno) derivado de los pericitos, que puede aparecer en cualquier parte del cuerpo; sin embargo, en el cuello se describen casos aislados. La resección quirúrgica completa constituye la piedra angular del tratamiento. Objetivo: Presentar un caso de un hemangiopericitoma en el cuello, como un caso inusual, con potencial maligno desconocido, diagnóstico y tratamiento oportuno. Presentación de caso: Paciente de sexo masculino, de 39 años de edad, sin antecedentes de enfermedad conocidos, con una masa perceptible a nivel V del cuello derecho. Estudios de imagen muestran un tumor vascularizado de aproximadamente 6 x 7 x 6 cm, entre los músculos escalenos, que fue originado en la arteria cervical profunda. Se confirmó mediante biopsia incisional el hemangiopericitoma, el cual fue tratado mediante resección tumoral completa y radioterapia adyuvante. Actualmente el paciente no tiene actividad tumoral después de su tratamiento inicial. Conclusiones: El hemangiopericitoma en el cuello es raro, el diagnóstico constituye un reto clínico e histológico, ya que, al ser poco común, su potencial maligno resulta desconocido. Aquellos tumores que tienen bajo grado de malignidad pueden ser controlados, de acuerdo a su localización y tamaño, mediante resección completa; mientras que los tumores de alto grado pueden recurrir y dar origen a metástasis. Nuestro paciente tuvo características histopatológicas con invasión capsular, lo que trajo como consecuencia un incremento del riesgo de recurrencia local. Por ese motivo, se decidió aplicar tratamiento adyuvante con radioterapia. El paciente se mantiene sin recurrencia tumoral local y a distancia después de 9 años de vigilancia médica(AU)

Introduction: Hemangiopericytoma is a rare mesenchymal tumor (vascularized and potentially malignant) derived from pericytes. It can occur anywhere in the body; however, isolated cases are described in the neck. Complete surgical resection is the cornerstone of treatment. Objective: To present a case of hemangiopericytoma in the neck, as an unusual case, with unknown malignant potential, as well as its timely diagnosis and treatment. Case presentation: A 39-year-old male patient, with no known history of disease, had a noticeable mass at the V level of the right neck. Imaging studies showed a vascularized tumor of approximately 6 x 7 x 6 cm, between the scalene muscles, which originated in the deep cervical artery. Hemangiopericytoma was confirmed by incisional biopsy, as well as treated by complete tumor resection and with adjuvant radiotherapy. Currently, the patient has no tumor activity after his initial treatment. Conclusions: Hemangiopericytoma in the neck is rare. Its diagnosis is a clinical and histologic challenge because, being uncommon, its malignant potential is unknown. Those tumors with low-grade malignancy can be controlled, according to their location and size, by complete resection; while high-grade tumors may recur and give rise to metastases. Our patient had histopathologic features with capsular invasion, which resulted in an increased risk of local recurrence. For this reason, adjuvant treatment with radiotherapy was decided to be applied. The patient remains without local or distant tumor recurrence after 9 years of medical surveillance(AU)

EGFR, VEGF, and angiogenesis promote the development of lipoma in the oral cavity

Abstract This study aimed to detect, quantify and compare the immunohistochemical expression of EGFR and VEGF and microvessel count (MVC) in oral lipomas, and to correlate the findings with clinical and morphological characteristics of the cases studied. The sample consisted of 54 oral lipomas (33 classic and 21 non-classic) and 23 normal adipose tissue specimens. Cytoplasmic and/or nuclear immunohistochemical staining of EGFR and VEGF was analyzed. The angiogenic index was determined by MVC. Cells were counted using the Image J® software. The Statistical Package for the Social Sciences was used for data analysis, adopting a level of significance of 5% for all statistical tests. A statistically significant difference in EGFR immunoexpression (p=0.047), especially, between classic lipomas and normal adipose tissue. There was a significant difference in MVC between non-classic lipomas and normal adipose tissue (p=0.022). In non-classic lipomas, only VEGF immunoexpression showed a significant moderate positive correlation (r=0.607, p=0.01) with MVC. In classic lipomas, the number of EGFR-immunostained adipocytes was directly proportional to the number of VEGF-positive cells, demonstrating a significant moderate positive correlation (r=0.566, p=0.005). The results suggest that EGFR, VEGF, and angiogenesis participate in the development of oral lipomas but are not primarily involved in the growth of these tumors.

Resumo Lipomas são as neoplasias mesenquimais benignas mais comuns, no entanto sua etiopatogenia ainda permanece desconhecida. Dessa forma, essa pesquisa teve como objetivo detectar, quantificar e comparar a expressão imunoistoquímica do EGFR, VEGF e contagem microvascular (MVC) dos lipomas orais, relacionando-os com as características clínicas e morfológicas dos casos estudados. A amostra foi composta por 54 lipomas orais (33 clássicos e 21 não clássicos) e 23 casos de tecido adiposo normal. A análise da expressão imunoistoquímica de EGFR e VEGF foi fundamentada na marcação citoplasmática e/ou nuclear. O índice angiogênico foi avaliado por meio da MVC. A contagem de células foi realizada utilizando software IMAGE J®. Os dados obtidos foram analisados no software Statistical Package for Social Science. O nível se significância de 5% foi adotado para os testes estatístico. A análise da imunoexpressão das proteínas revelou para o EGFR diferença estatisticamente significativa (p=0,041) entre o lipoma clássico e o tecido adiposo normal. Houve diferença significativa na MVC entre lipomas não clássicos e tecido adiposo normal (p=0,022). Nos lipomas não clássicos, apenas a imunoexpressão de VEGF apresentou correlação do tipo moderada, positiva e significativa (r=0,607; p=0,010) em relação a MVC. Ademais, nos lipomas clássicos foi percebido que os adipócitos imunomarcados para EGFR estiveram diretamente proporcionais a imunoexpressão de VEGF, apresentando correlação do tipo moderada, positiva e estatisticamente significativa (r=0,566; p = 0,005). Com base nos resultados, pode-se sugerir que o EGFR, VEGFR e MCV participam do desenvolvimento nos lipomas orais, contudo, não estão primariamente envolvidos no crescimento tumoral dessas neoplasias.

Angiomiolipoma hepático epitelioide: reporte de un caso / Hepatic epithelioid angiomyolipoma: Case Report

Angiomyolipomas (AML) are mesenchymal tumors belonging to the group of perivascular epithelioid cell tumors, which, rarely, can display a malignant behavior. They are composed of adipose tissue, vessels, and muscle tissue in different proportions, and constitute a differential diagnosis for other focal liver lesions. We report a 34-year-old woman in whom a focal hepatic lesion was discovered incidentally. The pathology report of an ultrasound guided biopsy informed an epithelioid angiomyolipoma, a rare variant of these lesions. During ten years of imaging follow, the size and features of the lesion has not changed. The patient rejected a surgical excision.

Intestinal Ewing sarcoma: An unusual presentation in the pediatric age / Sarcoma de Ewing intestinal: presentación inusual en la edad pediátrica

Abstract Background: Ewing's Sarcoma (ES) is the second most common type of bone cancer, with an annual incidence of 2.9:100,000. Extraosseous cases represent 15%; however, there are no reported cases of ES located in the intestine in the pediatric population. Case report: We describe the case of a 14-year-old male patient, previously healthy, who started with an anemic syndrome, weight loss, and diaphoresis of 8 weeks of evolution. After visiting a physician, who documented the presence of anemia, the patient was referred to the National Institute of Pediatrics. Physical examination showed grade III-IV systolic murmur, splenomegaly, and pain in the left hemiabdomen with no irradiation. Computed axial tomography showed a mass-dependent on the peritoneum and intestinal loop. A biopsy of the lesion showed intestinal ES. The lesion was completely resected, and the patient was treated with chemotherapy and radiotherapy. Thirty months after diagnosis, the patient has no evidence of tumor activity. Conclusions: Extraosseous presentation of ES in pediatric age is rare. There are no reports of intestinal ES in the Latin American pediatric population, although eight case reports were found in adults. ES is curable by a combination of chemotherapy, radiotherapy, and surgery. The medical literature indicates that the extraosseous presentation should receive the same treatment as the osseous presentation, which can provide a survival rate of up to 70% if there is no evidence of metastasis (which most frequently is observed in the lung).

Resumen Introducción: El sarcoma de Ewing (SE) es el segundo tipo cáncer más común de hueso, cuya incidencia anual es de 2.9:100,000. Los casos extraóseos representan el 15%; sin embargo, no existen reportes en la literatura de casos de SE ubicados en el intestino en la población pediátrica. Caso clínico: Se describe el caso de un paciente de sexo masculino de 14 años, previamente sano, que inició con síndrome anémico, pérdida de peso y diaforesis de 8 semanas de evolución. Acudió con un médico, quien documentó la presencia de anemia y lo refirió al Instituto Nacional de Pediatría. A la exploración física presentaba soplo sistólico grado III-IV, esplenomegalia y dolor en hemiabdomen izquierdo sin irradiaciones. La tomografía axial computarizada mostró una masa dependiente del peritoneo y asa intestinal. La biopsia de la lesión reportó SE intestinal. Se resecó por completo la lesión y el paciente recibió tratamiento con quimioterapia y radioterapia. Después de 30 meses del diagnóstico, el paciente se encuentra sin datos de actividad tumoral. Conclusiones: La presentación extraósea del SE en edad pediátrica es rara. No existen reportes de presentación de SE intestinal en la población pediátrica latinoamericana, aunque se encontraron ocho reportes de caso en adultos. El SE es curable mediante la combinación de quimioterapia, radioterapia y cirugía. La literatura médica indica que la presentación extraósea debe recibir el mismo tratamiento que la ósea, lo cual puede proporcionar una sobrevida de hasta el 70% si no hay evidencia de metástasis (que ocurre más frecuentemente a pulmón).

Phosphaturic mesenchymal tumor: An underdiagnosed rare entity

Tumor-induced osteomalacia is a paraneoplastic syndrome resulting in renal phosphate wasting and decreased bone mineralization. Phosphaturic mesenchymal tumors represent a rare etiology of tumor-induced osteomalacia. They are exceptionally rare, probably accounting for < 0.01% of all soft tissue tumors. Most PMTs present as small inapparent lesions that require very careful clinical examination and radionucleotide scan for localization. Here we describe a case in a 65 years old woman with recurrent multiple bone fractures and subsequent detection of a tumor involving right femur and adjacent soft tissue, low phosphate level and elevated serum Fibroblast growth factor-23 (FGF-23).

Leiomioma vesical. A propósito de un caso / Bladder leiomyoma. A case report

RESUMEN Fundamento: El leiomioma vesical es un tumor de origen mesenquimal, benigno, derivado de las fibras del músculo liso, su diagnóstico definitivo es por estudio histológico; su tratamiento es quirúrgico con pronóstico excelente. Son pocos los casos que se presentan en la bibliografía consultada por la escasa incidencia de este tipo de tumor. Objetivo: Reportar un caso diagnosticado con un leiomioma vesical. Presentación del caso: Se presenta el caso de una paciente femenina de 48 años de edad, atendida en el Hospital General Provincial Camilo Cienfuegos de Sancti Spíritus, que fue diagnosticada con ureterocele izquierdo. La paciente a los 7 años acudió con síntomas de polaquiuria, goteo posmiccional y sensación de repleción después de la micción. A la exploración física sin alteraciones en general. Ecografía renovesical con imagen quística en uréter distal izquierdo, de gran tamaño, con paredes gruesas, que ocupa casi la totalidad de la vejiga, con ureterohidronefrosis severa izquierda. Se le realizó cirugía definitiva, cistectomía parcial con reimplante ureteral izquierdo, con buena evolución. El resultado del diagnóstico anatomopatológico fue un leiomioma vesical. Conclusiones: El leiomioma vesical es una neoplasia poco frecuente en vejiga; en el caso reportado al realizarle el tratamiento quirúrgico se observó un excelente pronóstico.

ABSTRACT Background: Bladder leiomyoma is a benign tumor of mesenchymal origin, derived from smooth muscle fibers, its definitive diagnosis is by histological study; its treatment is surgical with excellent prognosis. Few cases are presented in the literature due to the low incidence of this nature. Objective: To report a case diagnosed with a bladder leiomyoma. Case report: A 48-year-old female patient, assisted at Camilo Cienfuegos General Provincial Hospital in Sancti Spíritus, diagnosed with left ureterocele. The patient came to consulting 7 years after have presented symptoms of pollakiuria, post-mictional drip and repletion sense afterwards urination. Physical examination showed no general alterations. Renovesical ultrasound showed a cystic image in the left distal ureter, large, thick-walled, occupying almost the entire bladder, with severe left ureterohydronephrosis. Definitive and partially cystectomy surgery was performed with left ureteral reimplantation, presenting good evolution. The result of the anatomopathological diagnosis was a bladder leiomyoma. Conclusions: Bladder leiomyoma is a rare neoplasm in the bladder; in the reported case, after surgical treatment, an excellent prognosis was observed.

Lipoma Cervicofacial Gigante: Reporte de un caso / Giant Cervicofacial Lipoma: A Case Report

RESUMEN: Los lipomas son neoplasias benignas formadas por tejido adiposo maduro y que representan los tumores mesenquimales más frecuentes. Los lipomas gigantes se definen en relación a un tamaño mayor de 10 centímetros o un peso mayor a 1 kilogramo. Su presentación a nivel de cabeza y el cuello es infrecuente. Se caracterizan por tener un crecimiento lento, progresivo, deformante y asintomático. Es necesaria la realización de pruebas de imagen para su correcta localización anatómica y establecer relaciones con las estructuras vecinas. El tratamiento de elección es la escisión quirúrgica de la masa. La planificación pre-quirúrgica en este tipo de casos debe ser minuciosa para garantizar la conservación tanto de la función como de la estética cervicofacial. Se reporta el caso de una mujer de 50 años que presenta una tumoración gigante cervicofacial de 9 años de evolución, diagnosticada como lipoma, con sus características clínicas, imageneológicas, histopatológicas y su manejo terapéutico.

ABSTRACT: Lipomas are benign neoplasms formed by mature adipose tissue and represent the most frequent mesenchymal tumors. Giant lipomas are defined in relation to a size greater than 10 centimeters or a weight greater than 1 kilogram. Their presentation at head and neck level is infrequent. They are characterized by having a slow, progressive, deforming and asymptomatic growth. Imaging tests are necessary for their correct anatomical location and the establishment of relationships with neighboring structures. The treatment of choice is surgical excision of the mass. Pre-surgical planning in this type of case must be meticulous to guarantee the preservation of both function and cervicofacial aesthetics. The case of a 50-year-old woman with a giant cervicofacial tumor of 9 years of evolution, diagnosed as lipoma, with its clinical, imaging, histopathological characteristics, and therapeutic management is reported.

Cellular angiofibroma: A rare vulvar tumor case report angiofibroma celular: Um caso raro de tumor vulvar

Abstract Cellular angiofibroma (CA)is a rare benign mesenchymal tumor. In women, it occurs mainly in the vulvovaginal region, with vulvar location in 70% of the cases. Its clinical presentation is nonspecific and similar to several other vulvar tumors of different cellular origins. Thus, its histological and immunohistochemical features allow distinction fromother tumors. Cellular angiofibromas have good prognosis, despite some risk of relapse. The authors present the case of a 49-year-old woman with a bulky right vulvar lesion, for which the preoperative diagnosis was a Bartholin cyst, but the histological and immunohistochemical evaluation yielded a CA.

Resumo O angiofibroma celular é um tumor mesenquimatoso benigno e raro. Nasmulheres, surge principalmente na região vulvo-vaginal, com localização vulvar em 70% dos casos. A sua apresentação clínica é inespecífica e semelhante a vários outros tumores vulvares de diferentes origens celulares. Assim, são as suas características histológicas e imunohistoquímicas que permitem a diferenciação entre eles. Os angiofibromas celulares têm bom prognóstico,embora apresentemalgumrisco de recidiva. Os autores apresentamo caso de umamulher de 49 anos de idade comuma lesão vulvar direita volumosa, cujo diagnóstico pré-operatório era de quisto da glândula de Bartholin, mas cujo exame histológico e imunohistoquímico revelaram tratar-se de um angiofibroma celular.

Tumor-Induced Osteomalacia: A Case Study Of A Nasal Tumor

Oncogenous osteomalacia, which is also known as tumor-induced osteomalacia, is a condition where a neoplasm isassociated with systemic bone demineralization and renal phosphaturia. We report a case who presented with a bleedingnasal mass, generalized fatigue, and cramps in her leg. Excision was done, and histopathologically, it was diagnosed tobe a phosphaturic mesenchymal tumor. The cramps disappeared soon after surgery and she is on regular follow-up forthe past 2 years.

Clinical and immunohistopathologic study of phosphaturic mesenchymal tumor / 中华病理学杂志

Objective@#To study the clinicopathological characteristics and immunohistochemical phenotype of phosphaturic mesenchymal tumor (PMT) .@*Methods@#The clinicopathological data and immunohistochemical profiles were obtained retrospectively from 206 patients diagnosed with PMT at Peking Union Medical College Hospital (PUMCH) during July 2008 to September 2017, with a review of literature.@*Results@#The mean age of PMT patients was 42 years (range 13 to 70 years), with a male to female ratio of 1.1∶1.0. All patients presented with different degree of bone pain, muscle weakness, shorten of stature, thoracic deformity and pathological fractures, with hypophosphatemia and high serum ALP. Phosphatemia returned to normal within 1 week after operation in all cases underwent complete tumor resection. The duration of osteomalacia before resection (documented in 197 cases) ranged from 20 days to 40 years (average 5.7 years). The average blood phosphorus concentration raised from 0.49 mmol/L to 0.92 mmol/L before and after tumor resection (P<0.01), with 147 cases (84.0%, 147/175) returned to normal range within 2 weeks. The rate or blood phosphorus concentration recovery in 15 days after operation was 79.6% in average, displayed significant differences between patients with complete resection and those with partial resection (85.4% vs. 21.1%, P<0.01). PMT lesions mainly involved lower extremities (55.8%), followed by head and neck (29.1%). In immunohistochemical study, all cases were positive for vimentin (100.0%), while most cases were positive for NSE (96.3%), CD56 (94.2%), FGF23(88.4%), CD68 (88.3%), D2-40 (70.9%), CD34 (23.1%), SMA (55.5%), bcl-2 (59.8%) and CD99 (47.1%). The Ki-67 positive index of tumor varied from less than 2% (51.4%), 3% to 10% (41.3%) to >10% (7.2%).@*Conclusions@#PMT mainly occurs in lower limbs or head and neck, with unique clinical characteristics and blood biochemical indexes. The tumor expresses a variety of immunohistochemical markers, indicating the potential of multi-directional differentiation. Clinical profile, blood biochemistry testing and immunohistochemical phenotype is helpful for diagnosis of PMT.

Successful Localization Using ⁶⁸Ga-DOTATOC PET/CT of a Phosphaturic Mesenchymal Tumor Causing Osteomalacia in a Patient with Concurrent Follicular Lymphoma / 대한핵의학회잡지

Diagnosing tumor-induced osteomalacia is often challenging because conventional imaging modalities may fail to locate the responsible tumor. This report describes the ability of ⁶⁸Ga-DOTATOC PET/CT to successfully distinguish between the responsible phosphaturic mesenchymal tumor and concurrent lymphoma lesions. A 52-year-old man with bone pain for several years was diagnosed with a vitamin D-resistant hypophosphatemic osteomalacia. Whole body ¹⁸F-FDG PET/CT revealed multiple enlarged hypermetabolic lymph nodes in his bilateral cervical, axillary, mediastinal, abdominal, pelvic, and inguinal regions. Core needle biopsy of the right cervical lymph node confirmed the diagnosis of follicular lymphoma. However, lymphoma was not considered the cause of osteomalacia. ⁶⁸Ga-DOTATOC PET/CT before chemotherapy showed a small nodule with intensely increased uptake in the right inguinal region, which was distinguished from the other enlarged lymph nodes. The nodule was surgically removed and histopathologically consistent with phosphaturic mesenchymal tumor. After surgery, the patient's serum phosphorus and alkaline phosphatase levels normalized without nutritional supplement.

Plexiform Angiomyxoid Myofibroblastic Tumor of the Stomach: a Rare Case

Plexiform angiomyxoid myofibroblastic tumor (PAMT) of the stomach is a very rare mesenchymal tumor of the gastrointestinal tract. We report a case of asymptomatic gastric PAMT that was pathologically confirmed after surgical resection. The tumor had a multinodular plexiform growth pattern, bland-looking spindle cells, and an Alcian blue-positive myxoid stromal matrix rich in small blood vessels. Immunohistochemistry analysis revealed that the tumor cells of the PAMT were positive for smooth muscle actin (SMA) and negative for c-kit, CD34, S-100 protein, epithelial membrane antigen (EMA), and desmin. PAMT should be differentiated from other submucosal tumors of the stomach by immunohistochemical findings. Considering the benign features of this tumor, observation without resection may be an option for the treatment of PAMT if the tumor is asymptomatic.

Vascular Leiomyosarcoma of the Thigh: A Rare Presentation.

High-grade mesenchymal soft tissue tumors are rare neoplastic lesions that occur uncommonly in the extremities and in other sites of the body. Herein, we report a case of high-grade vascular leiomyosarcoma of the thigh. The patient was a 72-year-old male with a 4-day history of swelling in his right thigh. Excision biopsy of the tumor showed highly pleomorphic tumor cells arranged typically in perivascular fashion with large areas of hemorrhage and necrosis. Immunohistochemistry was positive for vimentin, smooth muscle actin, and showed a 90% proliferation index on ki-67 labeling. Vascular leiomyosarcomas comprise a group of very infrequent tumors with varied presentation and can occur at unusual sites.

Solitary Fibrous Tumor of Head & Neck Region: Problems in Diagnosis Due to “Borderline” Features.

Aims: To report SFT in a 45 years male who had a lobulated tumor in neck for longest duration of 18 years with borderline histomorphology. Presentation of Case: The case discussed is of a forty-five year old Indian man who presented with painless mass in right side of neck for the duration of 18 years with rapid enlargement in last one year. Discussion: Solitary fibrous tumor is an unusual spindle cell neoplasm arising from primitive mesenchymal cell with distinct “patternlesss pattern” on histomorphology. The commonest site for this tumor is pleura but in recent years it has been described in various anatomic sites and is known to involve any region of the body. The extra- pleural sites include extremities, pelvis, head and neck and urogenital region. SFT of Head and Neck region is rare and usually involves deep soft tissues. Morphologically SFT resembles many benign and malignant soft tissue tumors. Conclusion: SFT is a rare tumor in neck region and has to be differentiated meticulously from other common and rare soft tissue tumors. Close follow up is essential after complete surgical excision in cases with “borderline” histomorphology.

Endosonographic Features of Gastric Schwannoma: A Single Center Experience

BACKGROUND/AIMS: Gastric schwannomas are rare benign mesenchymal tumors that are difficult to differentiate from other mesenchymal tumors with malignant potential, such as gastrointestinal stromal tumors. This study aimed to evaluate the characteristic findings of gastric schwannomas via endoscopic ultrasonography (EUS). METHODS: We retrospectively reviewed the EUS findings of 27 gastric schwannoma cases that underwent surgical excision at Pusan National University Hospital during 2007 to 2014. RESULTS: Gastric schwannomas were mainly located in the middle third of the stomach with a mean tumor size of 32 mm. All lesions exhibited hypoechoic echogenicity, and 24 lesions (88.9%) exhibited heterogeneous echogenicity. Seventeen lesions (63.0%) exhibited decreased echogenicity compared to the normal proper muscle layer. Distinct borders were observed in 24 lesions (88.9%), lobulated margins were observed in six lesions (22.2%), and marginal haloes were observed in 24 lesions (88.9%). Hyperechogenic spots were observed in 21 lesions (77.8%), calcifications were observed in one lesion (3.7%), and cystic changes were observed in two lesions (7.4%). CONCLUSIONS: During EUS, gastric schwannomas appear as heterogeneously hypoechoic lesions with decreased echogenicity compared to the normal proper muscle layer. These features may be helpful for differentiating gastric schwannomas from other mesenchymal tumors.

Resección laparoscópica de tumor del estroma gástrico / Laparoscopic resection of a gastric stromal tumor

Los tumores estromales gastrointestinales son los tumores mesenquimales más frecuentes del tracto digestivo. La mayor prevalencia de esta enfermedad se presenta en el estómago. El tratamiento de elección es la resección del área tumoral con márgenes histológicos sin linfadenectomia. Las técnicas de mínimo acceso muestran una alternativa muy favorable para el tratamiento de los tumores estromales gastrointestinales. Existen reportes aislados en la literatura de estos tumores tratados quirúrgica y oncológicamente. Por la escasa frecuencia con que se presenta la patología y los beneficios que brinda el acceso laparoscópico para su tratamiento, hemos realizado el presente trabajo con el objetivo de describir los aspectos clínicos del diagnóstico y tratamiento de un paciente que presenta tumor de páncreas versus tumor estromal gástrico. Se realiza laparoexploración donde se constata tumor de fundus gástrico, curvatura mayor y realiza resección tumoral laparoscópica. Con evolución favorable, confirmación inmunohistoquímica de tumores estromales gastrointestinales y seguimiento oncológico(AU)

Gastrointestinal stromal tumors are the most frequent mesenchymal tumors of the digestive tract. The highest prevalence of the disease is found in the stomach. The treatment of choice is resection of the tumor area with histological borders without lymphadenectomy. The minimal access techniques represent a very favorable alternative to treat gastrointestinal stromal tumors. There are few reports in literature about surgically and oncologically treated tumors of this type. The uncommon occurrence of this pathology and the benefits of laparoscopic techniques for treatment prompted us to present this paper in order to describe the clinical aspects of diagnosis and treatment of a patient who presented with pancreas tumor vs gastric stromal tumor. A laparoscopic examination was made to confirm the presence of a gastric fundus tumor and greater curvature, so laparoscopic tumor resection was performed, with favorable evolution, immunohistochemical confirmation of gastrointestinal stromal tumors and oncological follow-up(AU)

Osteomalacia oncogénica: Presentación de dos casos

La osteomalacia oncogénica es una enfermedad rara. Existen descriptos alrededor de 337 casos. Es ocasionada por un tumor productor del factor de crecimiento fibroblástico 23 (FGF-23), hormona que disminuye la reabsorción tubular de fosfatos y altera la hidroxilación renal de la vitamina D, con hipofosfatemia, hiperfosfaturia y niveles bajos de calcitriol. Se presentan dos pacientes de 44 y 70 años, que consultaron por dolores óseos generalizados de aproximadamente un año de evolución en los que se hallaron alteraciones bioquímicas compatibles con osteomalacia hipofosfatémica. En el primer caso se realizó la resección de una tumoración en tejido celular subcutáneo del pie derecho, un año después del diagnóstico clínico. Luego de la exéresis, se disminuyó el aporte de fosfatos que recibía el paciente, pero reaparecieron los dolores al intentar suspenderlos. Ocho años más tarde, hubo recidiva local de la tumoración por lo que se efectuó resección completa. Después de la misma, se logró suspender el aporte de fosfatos. En el segundo caso, el paciente se estudió con tomografía por emisión de positrones con 18F-fluorodesoxiglucosa, hallando formación nodular hipermetabólica en partes blandas de antepie derecho, de 2.26 cm de diámetro. Luego de su escisión se pudo suspender el aporte de fosfatos. Ambos pacientes se encuentran asintomáticos con indicadores de metabolismo fosfocálcico normales. El diagnóstico anatomopatológico en ambos fue un tumor mesenquimático fosfatúrico, variante mixta del tejido conectivo, la entidad más frecuentemente asociada a la osteomalacia oncogénica.

Oncogenic osteomalacia is a rare disease. It is caused by a tumor that produces fibroblast growth factor 23, a hormone that decreases the tubular phosphate reabsorption and impairs renal hydroxylation of vitamin D. This leads to hyperphosphaturia with hypophosphatemia and low calcitriol levels. About 337 cases have been reported and we studied two cases; 44 and 70 year-old men who sought medical attention complaining of suffering diffuse bone pain over a period of approximately one year. In both cases, a laboratory test showed biochemical alterations compatible with a hypophosphatemic osteomalacia. In the first case, a soft tissue tumor of the right foot was removed, one year after the diagnosis. The patient was allowed to diminish the phosphate intake, but symptoms reappeared at this time. Eight years later, a local recurrence of the tumor was noted. A complete excision was now performed. The patient was able to finally interrupt the phosphate intake. In the second case, an F-18 fluorodeoxyglucose positron emission tomography, with computed tomography revealed a 2.26 cm diameter hypermetabolic nodule in the soft tissue of the right forefoot. After its removal, the patient discontinued the phosphate intake. Both patients are asymptomatic and show a regular phosphocalcic laboratory evaluation. The histopathological diagnosis was, in both cases, a phosphaturic mesenchymal tumor, a mixed connective tissue variant. This is the prototypical variant of these tumors.

Hyperphosphaturic Mesenchymal Tumor induced Osteomalacia– A Case Report.

Hyperphosphaturic Mesenchymal Tumor (HMT) is a very rare benign tumor of the soft tissue or bone which produces tumor induced osteomalacia, also called as oncogenic osteomalacia. This activity can only be stopped by the surgical removal of the tumor. We present a 23 years old man who presented with long standing bony pains without any relief by a variety of medications. The clue to the diagnosis was taken from pelvis skiagram, Magnetic Resonance Imaging (MRI) of the body, PET scan and the blood chemistry.