Fibrocartilaginous mesenchymoma: a clinicopathological analysis of four cases / 中华病理学杂志

Objective: To investigate the clinical, radiological, histological and molecular features and the differential diagnosis of fibrocartilaginous mesenchymoma (FM). Methods: Four cases of FM diagnosed in the Department of Pathology, the Sixth People's Hospital Affiliated to Shanghai Jiaotong University School of Medicine from 2020 to 2022 were analyzed. Related literature was also reviewed. Results: Case 1 was a 10-year-old girl with bone destruction in the sacrum and L5 articular processes revealed by CT scan. Case 2 was a 7-year-old girl with an aggressive lesion in her right distal ulna. Case 3 was an 11-year-old boy with a lesion in the metaphysis of his left proximal tibia. Case 4 was an 11-year-old boy with bone destruction in the distal portion of a radius. Microscopically, the four tumors all consisted of numerous spindle cells, hyaline cartilage nodules, and bone trabeculae. The hypocellular to moderately cellular spindle cell component contained elongated cells with slightly hyperchromatic, mildly atypical nuclei arranged in bundles or intersecting fascicles. Benign-appearing cartilaginous nodules of various sizes and shapes were scattered throughout the tumors. There were areas mimicking epiphyseal growth-plate characterized by chondrocytes arranged in parallel columns and areas of enchondral ossification. The stroma was rich in mucus in case 1. Mutation of GNAS and IDH1/IDH2 and amplification of MDM2 gene were not found in any of the three tested cases. Conclusions: FM is very rare and tends to affect young patients. It most frequently occurs in the metaphysis of long tubular bones, followed by the iliac-pubic bones and vertebrae. FM is characterized by a mixed population of spindle cells, hyaline cartilage nodules and trabeculae of bone, without specific immunophenotypes and molecular alternations. As a borderline, locally aggressive neoplasm, surgical removal with a wide margin is generally the treatment of choice for FM.

Tumor mesenquimático fosfatúrico de pelvis: abordaje multidisciplinario / Phosphaturic mesenchymal tumor of the pelvis: A multidisciplinary approach

El tumor mesenquimático fosfatúrico es una entidad clinicopatológica sumamente infrecuente. Además de provocar dolor óseo insidioso y polimialgias, se acompaña de alteraciones del metabolismo fosfocálcico de difícil manejo clínico. El abordaje multidisciplinario resulta la clave del éxito en esta enfermedad. Presentamos una paciente de 52 años de edad con antecedente de tumor mesenquimático fosfatúrico en la hemipelvis derecha con extensión a la cadera homolateral de 10 años de evolución. Clínicamente presentaba osteomalacia oncogénica (hipofosfatemia e hiperfosfaturia) que no se corregía, pese a un agente de última generación, el burosumab, un inhibidor del factor de crecimiento fibroblástico 23, que aumenta la reabsorción tubular renal de fosfatos. En un comité multidisciplinario, se decidió la resección con márgenes oncológicos y se logró una mejoría clínica franca. Comunicamos este caso, debido a que es un cuadro infrecuente. Nivel de Evidencia: IV

Phosphaturic mesenchymal tumor (PMT) is an infrequent clinicopathological entity. It presents insidious bone pain and polymyalgia, accompanied by alterations in calcium and phosphorus metabolism that are difficult to resolve clinically. A multidisciplinary approach is a key to success in this pathology. We present the case of a 52-year-old female patient with a 10-year history of PMT in the right hemipelvis with ipsilateral hip extension. From the clinical point of view, she presented oncogenic osteomalacia (hypophosphatemia and hyperphosphaturia) that did not correct despite being administered the latest generation medication, burosumab, an FGF-23 inhibitor that increases renal tubular phosphate reabsorption. Resection with oncological margins was decided by a multidisciplinary committee resolving her clinical condition. Due to the rarity of this pathology, we decided to report the case. Level of Evidence: IV

Diagnosis and surgical treatment of sinonasal phosphaturic mesenchymal tumor / 中华耳鼻咽喉头颈外科杂志

Objective: To investigate the diagnosis and surgical treatment of sinonasal phosphaturic mesenchymal tumor (PMT). Methods: The medical records of nine patients who had been diagnosed as sinonasal PMT in Department of Otorhinolaryngology Head and Neck Surgery, Shanghai JiaoTong University Affiliated Sixth People's Hospital between January 2015 and May 2020 were collected, including 4 males and 5 females, ranging from 36 to 59 years. The patient's previous history, clinical manifestations, imaging findings, laboratory results, surgical procedure, pathological results and postoperative follow-up data were analyzed by descriptive statistical analysis. Results: All patients presented hypophosphatemia and tumor-induced osteomalacia (TIO) with a disease course of 1 to 19 years. The imaging examination and intraoperative findings identified two cases with peripheral tissue infiltration, two cases with contralateral nasal cavity invasion, and one case with intracranial invasion. Five patients underwent unilateral endoscopic resection while two patients underwent bilateral endoscopic resection, and the remaining two patients underwent unilateral transorbital ethmoid artery ligation plus endoscopic tumor resection and endoscopic combined with transfrontal tumor resection (n=1 each). Expect for one case developed recurrence and intracranial involvement, the other patients achieved clinical remission and no recurrence was observed during the six-month follow-up. Conclusions: The diagnosis of sinonasal PMT needs combination of clinical manifestation, imaging, and pathological findings. Complete surgical excision and long-term postoperative follow-up are imperative.

Angiomixoma agresivo postrasplante renal / Aggresive angiomyxoma after renal transplantation

La condición de inmunosuprimido aumenta el riesgo de cáncer en trasplantados renales, en comparación a la población general. La mejor supervivencia de esta población en los últimos años ha convertido a las neoplasias y a la enfermedad cardiovascular en las principales causas de morbi-mortalidad. Presentamos el caso de un paciente trasplantado renal que desarrolló cuatro años después del trasplante una forma inusual de tumor mesenquimatoso, el angiomixoma agresivo, que requirió resección quirúrgica amplia.

The condition of immunosuppressed increases the risk of cancer in kidney transplant patients, as compared to the general population. The best survival of inmunosupressed patients in recent years has turned both neoplasms and cardiovascular diseases into the main causes of morbidity and mortality. We present the case of a renal transplanted patient who developed an unusual form of mesenchymal tumor such as the aggressive angiomyxoma, four years after the implant and requiring wide surgical resection.

Ectomesenchymal chondromyxoid tumor: a comprehensive updated review of the literature and case report / 国际口腔科学杂志·英文版

Prompted by a unique case of an ectomesenchymal chondromyxoid tumor (ECT) of the palate in a 54-year-old female, we reviewed the English and German literature on this entity until the end of 2016 using PubMed. The search produced 74 lingual cases with a nearly equal sex distribution and a mean age of 39.3 years, and two extra-lingual cases sharing histological and immunohistological features including nodular growth, round, fusiform or spindle-shaped cellular architecture, and chondromyxoid stroma. Immunophenotyping showed the majority of cases to be positive for glial fibrillary acidic protein (GFAP), S-100 protein, glycoprotein CD57, pancytokeratin (AE1/AE3), and smooth muscle actin (SMA); in isolated cases there was molecular-genetic rearrangement or gain of Ewing sarcoma breakpoint region 1 (EWSR1) but no rearrangement of pleomorphic adenoma gene 1 (PLAG1). At present, ectomesenchymal cells that migrate from the neural crest are considered to play a pivotal role in tumor origin. All cases had a benign course, although there were three recurrences. Because of the rarity of this tumor and the need for differential diagnostic differentiation from myoepithelioma and pleomorphic adenoma, both oral surgeons and pathologists should be aware of this entity.

Endoscopic Ultrasonography in the Diagnosis and Treatment Strategy Choice of Esophageal Leiomyoma

OBJECTIVES: Esophageal leiomyoma is the most common benign tumor of the esophagus, and it originates from mesenchymal tissue. This study analyzed the clinicopathological characteristics of esophageal leiomyoma and aimed to evaluate the role of endoscopic ultrasonography in the diagnosis and treatment selection for these lesions. METHODS: Two hundred and twenty-five patients who had suspected esophageal leiomyomas in endoscopic ultrasonography were enrolled at the Endoscopy Center of The First Affiliated Hospital, Zhejiang University from January 1st, 2009 to May 31th, 2015. The main outcomes included the demographic and morphological characteristics, symptoms, comparisons of diagnosis and treatment methods, adverse events, and prognosis. RESULTS: One hundred and sixty-seven patients were diagnosed as having an esophageal leiomyoma by pathological examination. The mean patient age was 50.57±9.983 years. In total, 62.9% of the lesions originated from the muscularis mucosa, and the others originated from the muscularis propria. The median distance to the incisors was 30±12 cm. The median diameter was 0.72±0.99 cm. As determined by endoscopic ultrasonography, most existing leiomyomas were homogeneous, endophytic, and spherical. The leiomyomas from the muscularis mucosa were smaller than those from the muscularis propria and much closer to the incisors (p<0.05). SMA (smooth muscle antibody) (97.2%) and desmin (94.5%) were positive in the majority of patients. In terms of treatments, patients preferred endoscopic therapies, which led to less adverse events (e.g., intraoperative bleeding, local infection, pleural effusion) than surgical operations (p<0.05). The superficial leiomyomas presented less adverse events and better recovery (p<0.05) than deep leiomyomas. CONCLUSION: Endoscopic ultrasonography has demonstrated high accuracy in the diagnosis of esophageal leiomyomas and provides great support in selecting treatments; however, EUS cannot completely avoid misdiagnosis, so combining it with other examinations may be a good strategy to solve this problem.

Primary osteosarcoma of the breast: pathological and imaging findings / Osteossarcoma primário da mama: achados patológicos e de imagem

SUMMARY Primary osteosarcoma of the breast (POB) is an extremely rare and aggressive tumor. Differential diagnosis of POB includes osteosarcoma of the chest wall and metaplastic breast carcinoma. Imaging tests that exclude the existence of a direct connection between the tumor and chest wall, as well as histopathological and immunohistochemical studies that rule out the presence of an epithelial component are required for the diagnosis of POB. We report a case of a 69-year old woman with POB. Imaging and pathological findings are presented. Therapeutic approach is discussed in the light of current knowledge, including potential complications.

RESUMO O osteossarcoma primário da mama (OPM) é um tumor extremamente raro e agressivo. O diagnóstico diferencial do OPM inclui o osteossarcoma da parede torácica e o carcinoma metaplásico da mama. Exames de imagem que excluam a existência de uma conexão direta entre o tumor e a parede torácica, e estudos histopatológico e imuno-histoquímico que descartem a presença de um componente epitelial são necessários para o diagnóstico de OPM. Relatamos um caso de OPM em uma mulher de 69 anos de idade. Os achados de imagem e patológicos são apresentados. A abordagem terapêutica é discutida à luz do conhecimento atual, incluindo potenciais complicações.

Diagnosis and treatment of prostatic malignant mesenchymal tumors: Analysis of 20 cases / 中华男科学杂志

<p><b>OBJECTIVE</b>To explore the diagnosis, treatment, and prognosis of prostatic malignant mesenchymal tumors (PMMT).</p><p><b>METHODS</b>We retrospectively analyzed the clinical and follow-up data about 20 cases of PMMT and reviewed the literature relevant to the diagnosis, treatment, and prognosis of the disease.</p><p><b>RESULTS</b>Based on the results of pathology and immunohistochemistry, the 20 PMMT cases included leiomyosarcoma (n = 7), rhabdomyosarcoma (n = 5), prostatic stromal sarcoma (n = 3), chondrosarcoma (n = 1), and undifferentiated PMMT (n = 4). Twelve of the patients were treated by radical prostatectomy (3 concurrently by sigmoid colostomy and 1 by cystostomy), 2 by pelvic tumor resection following arterial embolization, 1 by total pelvic exenteration, 1 by colostomy with pelvic lymph node biopsy, and 4 by conservative therapy because of metastasis to the lung, pelvis and bone. Of the 20 patients, 9 died of systemic metastasis within 3 months after treatment, 3 died at 6, 7, and 14 months, respectively, 3 survived with tumor for 5, 11, and 12 months, respectively, 2 survived without tumor for 12 and 24 months so far, all subjected to periodic chemotherapy postoperatively, and 3 lost to follow-up.</p><p><b>CONCLUSION</b>PMMT is a tumor of high malignancy and rapid progression, for which transrectal ultrasound-guided biopsy remains the main diagnostic method. The clinical stage of the tumor is an important factor influencing its prognosis and the survival rate of the patients can be improved by early diagnosis and combined therapy dominated by radical prostatectomy.</p>

Malignant mesenchymal tumor of male breast: primary chondrosarcoma

Breast Sarcomas have relatively been rare and accounted for 1% of all primary malignant tumors of the breast. Pure and primary chondrosarcoma of the male breast would be an extremely rare tumor. It might arise either from the breast stroma itself, or from underlying bone or cartilage. A 65-year-old man has presented with a rapidly growing breast mass since 5 months. Physical examination has established a large firm to hard mass with regular margins in the region of right breast. There was no axillary lymphadenopathy. Contrast enhanced MRI of breasts has shown a mixed-signal intensity multi lobulated cystic-solid mass [10.4 cm × 10.3 cm ×9.9 cm] appearing predominantly hyper intense on T2W and hypo intense on T1W. The tumor has diagnosed as a low-grade chondrosarcoma of the breast by histopathological and immunohistochemistry analysis. Right sided radical mastectomy with grafting has done. It has seemed to be very important to identify the mammary primary sarcomas as entity separated from the carcinomas of the breast

Mesenquimoma fibrocartilaginoso de húmero proximal: reporte de un caso / Fibrocartilaginous mesenchymoma of the proximal humerus: case report

El mesenquimoma fibrocartilaginoso (MFC) es un tumor raro, que afecta principalmente a los huesos largos. Se han reportado pocos casos desde su descripción. Presentamos un caso de un niño de 4 años de edad con MFC situado en el húmero. Las radiografías mostraron una lesión lítica expansiva situada en la región metafisaria del húmero proximal. La resonancia magnética objetivó expansión del tumor a partes blandas. La anatomía patológica fue confirmatoria de MFC. El paciente fue tratado con curetaje, fenolización adyuvante y sustituto óseo mezclado con aspirado de médula ósea. A los dos años de seguimiento, no se evidenció recidiva. El MFC debe ser tenido en cuenta entre los diagnósticos diferenciales en lesiones óseas líticas en niños y adolescentes.

Fibrocartilaginous mesenchymoma (FCM) is a rare tumor that primarily affects the long bones. Few cases have been reported since its description. A case of a 4-year-old boy with FCM located in the humerus is presented. Radiological examination showed an expansive lytic lesion located in the metaphyseal proximal humerus. Magnetic resonance imaging showed soft tissue expansion. Histopathological diagnosis was confirmatory of FCM. Curettage, adjuvant phenolization, and bone grafting with bone substitute, and autologous bone marrow was performed. During a follow-up period of 2 years, there was no evidence of disease progression. FCM should be considered in the differential diagnosis of lytic bone lesions in children and adolescents.

Extracardial rhabdomyoma: a clinicopathologic analysis of 9 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic characteristics, differential diagnosis and biological behavior of extracardiac rhabdomyoma.</p><p><b>METHODS</b>Nine cases of extracardiac rhabdomyoma diagnosed between January of 1997 and July of 2014 were reviewed. The clinical, pathologic and immunohistochemical profiles were evaluated.</p><p><b>RESULTS</b>There were 5 males and 4 females at diagnosis with age ranging from 2 years and three months to 59 years (mean, 37.6 years). Sites included the head and neck region (7 cases), chest (1 case ) and vagina wall (1 case). Clinically, most cases manifested as a subcutaneous nodule or as a submucosal polypoid lesion with a mean diameter of 3.2 cm. Histologically, 4 were adult-type rhabdomyoma characterized by tightly packed large round or polygonal rhabdomyoblasts with abundant eosinophilic to clear cytoplasm; 3 were myxoid variant of fetal rhabdomyoma composed of immature myofibrils, spindled and primitive mesenchymal cells embedded in a myxoid background, 1 was an intermediate form of fetal rhabdomyoma consisting of densely arranged differentiated myoblasts with little myxoid stroma; 1 was a genital rhabdomyoma composed of elongated or strap-like myoblasts scattered in loose fibrous connective tissue. By immunohistochemistry, they showed diffuse and strong positivity for desmin, MSA and myoglobin with variable expression of myogenin. A case of intermediate type also stained for α-smooth muscle actin. Follow up data (2 months ~ 17 years) showed local recurrence in one patient 6 months after surgery.</p><p><b>CONCLUSIONS</b>Rhabdomyoma is a distinctively rare benign mesenchymal tumor showing skeletal muscle differentiation, which may occassionally recur if incompletely excised. Familiarity with its clinical and morphological variants is essential to avoid misdiagnosing this benign lesion as embryonal rhabdomyosarcoma.</p>

Tumor mesenquimatoso fosfatúrico de fosa nasal con compromiso intracraneano: reporte de un caso y revisión de la literatura / Nasal fossa phosphaturic mesenchymal tumor with intracraneal compromise: case report and review of the literature

El tumor mesenquimatoso fosfatúrico (TMF) es una enfermedad extremadamente rara. Según evidencia reciente es causado por la sobreexpresión del factor de crecimiento fibroblástico 23 (FGF23), el cual genera hipofosfemia y osteomalacia. A continuación presentamos el caso de un paciente de 42 años con un tumor mesenquimatoso fosfatúrico de fosa nasal izquierda con extenso compromiso intracraneano. Cabe destacar que hasta la fecha hay 142 casos reportados de TMF en la literatura de los cuales solo 11 se ubican en fosa nasaly cavidades sinusales, y sólo dos de ellos ubicados en fosa nasal¹. El paciente tuvo una exitosa resolución quirúrgica con la consecuente normalización de parámetros analíticos (incluido el FGF23), mejoría sintomática y ausenia de recidiva hasta la fecha.

The phosphaturic mesenchymal tumor (PMT) is an extremely rare disease. According to recent evidence is caused by overexpression of fibroblast growth factor 23 (FGF23) which generates hypophosphatemia and osteomalacia. We report the case of a 42 year old patient with a left nasal fossa phosphaturic mesenchymal tumor with intracranial involvement. Should be noted that to date there are 142 reported cases of PMT in the literature of which only 11 are located in nasal fossa and sinus cavities, two of them located in nasal fossa¹. The patient had a successful surgical resolution with consequent normalization of analytical parameters (including FGF23), absence of symptoms and no recurrence to date.

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.

Comparative of gastrointestinal mesenchymal tumors of CT virtual endoscopy and pathology / 中国医疗器械杂志

<p><b>OBJECTIVE</b>To observe the lower gastrointestinal mesenchymal tumors CT virtual endoscopy and pathology, and to explore its possible mechanism.</p><p><b>METHODS</b>Select 80 patients suspected colon gastroenterology treat in the hospital gastroenterology department, randomly divided into experimental group and control group, 40 cases in experimental group were given CT virtual endoscopy and pathological check, the control group received conventional colonoscopy and pathological check, and all the results compared with the pathological results.</p><p><b>RESULTS</b>Compared with the pathological test results, consistent rate with the experimental group reached 94.73%, the same rate was 92.85% in control group. The difference was not statistically significant (P > 0.05).</p><p><b>CONCLUSIONS</b>CT virtual endoscopy can quickly image and draw detailed information, and can improve the sensitivity and specificity of cancer diagnosis greatly. Clinically, CT virtual endoscopy have the same function as colonoscopy, can replace colonoscopy, can be important complement of electronic colonoscopy by those who cannot tolerate electronic colonoscopy or have contraindications. Its value is worth taking a step forward in-depth study.</p>

Leiomioma de ciego. Presentación de un caso / Leiomyoma of the cecum. A case report

Se presenta un caso de un paciente femenino de 51 años de edad con un leiomioma de ciego. Se le realizaron estudios complementarios y se diagnostico un tumor de anexo derecho con compromiso de la pared de ciego.

The case of a 51 year old female patient with a leiomyoma of the cecum is presented. Supplementary studies were performed and a right annex tumor with cecum wall involvement was diagnosed.

Tumor-induced osteomalacia with elevated fibroblast growth factor 23: a case of phosphaturic mesenchymal tumor mixed with connective tissue variants and review of the literature / 癌症

Tumor-induced osteomalacia (TIO), or oncogenic osteomalacia (OOM), is a rare acquired paraneoplastic disease characterized by renal phosphate wasting and hypophosphatemia. Recent evidence shows that tumor-overexpressed fibroblast growth factor 23 (FGF23) is responsible for the hypophosphatemia and osteomalacia. The tumors associated with TIO are usually phosphaturic mesenchymal tumor mixed connective tissue variants (PMTMCT). Surgical removal of the responsible tumors is clinically essential for the treatment of TIO. However, identifying the responsible tumors is often difficult. Here, we report a case of a TIO patient with elevated serum FGF23 levels suffering from bone pain and hypophosphatemia for more than three years. A tumor was finally located in first metacarpal bone by octreotide scintigraphy and she was cured by surgery. After complete excision of the tumor, serum FGF23 levels rapidly decreased, dropping to 54.7% of the preoperative level one hour after surgery and eventually to a little below normal. The patient's serum phosphate level rapidly improved and returned to normal level in four days. Accordingly, her clinical symptoms were greatly improved within one month after surgery. There was no sign of tumor recurrence during an 18-month period of follow-up. According to pathology, the tumor was originally diagnosed as "lomangioma" based upon a biopsy sample, "proliferative giant cell tumor of tendon sheath" based upon sections of tumor, and finally diagnosed as PMTMCT by consultation one year after surgery. In conclusion, although an extremely rare disease, clinicians and pathologists should be aware of the existence of TIO and PMTMCT, respectively.

Angiomixoma agresivo: presentación clínico patológica de caso y revisión de la literatura / Aggressive angiomyxoma: clinical pathological case presentation and review of literature

El angiomixoma agresivo es una neoplasia mesenquimal benigna, poco frecuente con predilección por la pelvis y periné femeninos, con tendencia a la recurrencia después de la excisión. Se presenta el caso de una mujer de 31 años con una tumoración polipoide en vulva. Histológicamente la tumoración presentaba estroma mixoide, hipocelular, vasculatura variable. Las células tenían aspecto miofibroblástico y su inmunofenotipo fue positivo para Desmina, Alfa Actina, Actina HHF-35 y receptor de estrógenos.

Aggressive angiomyxoma is a benign mesenchymal neoplasm, uncommon with a predilection for the female pelvis and perineum, with tendency to recur after excision. A case of a 31 year old woman with a polypoid tumor in the vulva. Histologically the tumor stroma showed myxoid, hypocellular, vasculature variable. Myofibroblastic cells had the appearance and the immunophenotype was positive for desmin, alpha actin, actin HHF-35 and estrogen receptor.

Tumores mesenquimales diagnosticados en la unidad de gastroenterología del HMPC entre los años 2007 aL 2009 / Mesenchymal tumors diagnosed in the gastroenterology unit of HPMC from 2007 to 2009

Se evaluaron las historias médicas con diagnóstico de tumores mesenquimales confirmados por análisis inmunohistoquímico entre los años 2007 y 2009. El objetivo fue describir a la población según características clínicas, epidemiológicas, diagnósticos por imágenes y análisis anatomopatológico. Se obtuvieron 11 pacientes con diagnósticos de GIST(7), Leiomiomas(2), GANT(1) y Leiomiosarcoma(1). Del género femenino (82%) y masculino (18%). Con un promedio de 55 años de edad. Clínicamente presentaron dolor abdominal (45%), mareos (27%), pirosis (9%) y disfagia (1%). El 18% permaneció asintomático. Entre los signos encontrados figuran: melena (36%), pérdida de peso (27%), palidez cutánea (9%) y vómitos (9%). El 36% de los pacientes no presentaron hallazgos al examen físico. Se realizó ultrasonido abdominal en el 100% de los pacientes, con hallazgos patológicos relacionados con el tumor en el 27 % de los casos. Al 90% de los pacientes se les realizó una Endoscopia Digestiva Superior, el 82% de los hallazgos se describieron como Tumores Submucosos y 9% se reportó como normal. Las lesiones se ubicaron en el estómago (60%), 27% en el intestino delgado ( duodeno y yeyuno); 9% en esófago y 9% en retroperitoneo. El ultrasonido endoscópico fue practicado al 82% de los pacientes, con un porcentaje de aciertos diagnósticos en el 90% de los tumores localizados en esófago, estómago e intestino delgado, y de 87,5% si se incluye el tumor retroperitoneal. La Tomografía Computada (TC) de abdomen fue practicada en 45% de los pacientes, con hallazgos patológicos en el 100% de los casos, descritos como Lesiones Ocupantes de Espacio (LOE), en estómago, intestino, esófago y retroperitoneo; y en el 20% de los pacientes se encontró enfermedad metastásica hepática Recibieron tratamiento quirúrgico el 82%, el 18% restante no se realizó por contraindicación médica o se encuentran en espera del procedimiento. Y un 18% de los pacientes recibieron tratamiento médico con Imatinib...

We evaluated the clinical records with a diagnosis of mesenchymal tumors confirmed by immunohistochemical analysis, from 2007 to 2009. The objective was to describe the population according to clinical and epidemiological features, diagnostic imaging and histopathological analysis. We found 11 patients with diagnoses of GIST(7), leiomyoma(2), leiomyosarcoma(1) and GANT(1); (82%) female and (18%) male, with a mean age of 55 years. They clinically presented abdominal pain (45%), dizziness (27%), heartburn (9%) and dysphagia (1%). 18% remained asymptomatic. Among the symptoms were: melena (36%), weight loss (27%), paleness (9%) and vomiting (9%). 36% of the patients had no findings at physical examination. Abdominal ultrasound was performed in 100% of the patients, with pathological findings related to the tumor in 27% of cases. In 90% of patients an upper digestive endoscopy was performed. 82% of findings were described as submucosal tumors and 9% was reported as normal. Lesions were located in stomach (60%); 27% in small bowel (duodenum and jejunum), 9% in esophagus and 9% in retroperitoneum. Endoscopic ultrasound was performed to 82% of patients, with a diagnostic accuracy of 90% for the tumors located in esophagus, stomach and small bowel; and 87,5% if the retroperitoneal tumor is included. The abdomen Computed Tomography (CT) was performed in 45% of patients with pathological findings in 100% of the cases, described as Space occupying lesion (SOL) in stomach, intestine, esophagus and retroperitoneum; and, in 20% of patients metastatic liver cancer was found. 82% received surgical treatment, the remaining 18% was not performed due to contraindication or are waiting for the procedure. And 18% of patients received medical therapy with Imatinib...