[Epithelioid uterine leiomyoma with giant cystic degeneration. A case report]. / Leiomioma uterino epitelioide con degeneración quística gigante. Reporte de un caso.

At present, show woman 46-years-old, presented hiperpolimenorrea, pelvis ultrasound appearance with cystic giant mass in the uterine corpus; histopathological revealed a epithelioid leiomyoma with giant cystic degeneration. We considered it is the first reported case of this variant of the epithelioid leiomyoma.

Epithelioid leiomyoma of the bladder: an unusual cause of voiding symptoms.

Epithelioid leiomyoma of the bladder is a rare benign neoplasm. A 63-year-old woman with a 2-year history of frequency and urgency was found to have a bladder mass on intravenous urography and subsequent cystoscopy. The mass was removed endoscopically. The patient's symptoms resolved.

Leiomyoma of the male urethra: a case report and review of the literature.

We describe the case of a 48-year-old quadriplegic black man with history of C4-C5 cervical spine and cord injury secondary to a fall, who presented to the University of Cincinnati Medical Center Urology Service with obstructive symptoms at urination. A bulbous urethral stricture was diagnosed and subsequently resected with primary urethral reanastomosis. On pathologic examination, the surgical specimen contained an epithelioid leiomyoma at the site of the urethral stricture. Although leiomyomas of the female urethra are relatively common, we identified only 2 previously reported cases of leiomyomas of the male urethra in the English-language medical literature. To the best of our knowledge, we describe the third case of leiomyoma of the male urethra, the first of the epithelioid type.

Leiomyoblastoma causing acute gastric outlet obstruction in an infant.

A 1-year-old boy had acute gastric outlet obstruction, the cause of which was a prepyloric submucosal mass. The mass and the ulcerated overlying mucosa were excised. The histological diagnosis was leiomyoblastoma with parenchymatous hemorrhage. Apparently, he is the youngest patient reported to have this disease.

Hemoperitoneum secondary to exogastric leiomyosarcoma: a case report.

We present the case of a 50-year-old man with hemoperitoneum secondary to a bleeding exogastric leiomyosarcoma. The patient underwent emergency laparotomy and resection of the mass en block with a segment of the lesser curvature was successfully performed.

An unusual cause of haemorrhagic ascites following blunt abdominal trauma.

Slow intraperitoneal haemorrhage following blunt abdominal trauma may present as haemorrhagic ascites. Such haemorrhage is usually due to rupture of spleen, liver or damage to small bowel mesenteric vasculature. We encountered a patient with bleeding from ruptured exogastric leiomyoblastoma. Two cases of traumatic rupture of gastric leiomyosarcomas have been reported previously. The operative treatment is usually delayed and the diagnosis established only at laparotomy. We suggest a high level of suspicion and early laparotomy.

[Pheochromocytoma-gastric leiomyoblastoma association. A possible expression of Carney's triad. A case report]. / Associazione feocromocitoma-leiomioblastoma gastrico. Possibile espressione di triade di Carney. Un caso di nostra osservazione.

The case of a 28 yrs old female patient affected by pheochromocytoma and gastric leiomyoblastoma was described. The simultaneous presence of these neoplasms in a young woman supported the diagnosis of the clinical syndrome known as "Carney's triad".

[Exceptional etiologies in upper digestive tract bleeding ]. / Exceptii etiologice în HDS.

7 cases, considered as being true etiological exceptions selected from 756 upper gastrointestinal bleeding, are presented. The causes of bleeding were: pancreatic pseudocyst with intracystic hemorrhage broken into duodenum (2 cases), the nonepithelial gastroduodenal tumor (3 cases), the aneurysm of gastroduodenal artery broken into duodenum (1 case) and the aortoduodenal fistula in one patient with a bilateral aorto-iliac by-pass (1 case). The etiological diagnosis could not be established in any cases before the operations. All the cases were operated on, the operation being imposed by the severity of bleeding and having the haemostasis as a main purpose.

Comparativa entre el abordaje abierto y el mínimamente invasivo en el tratamiento quirúrgico del leiomioma esofágico / Comparative study between open and minimally invasive approach in the surgical management of esophageal leiomyoma

Introducción: el leiomioma es el tumor benigno más frecuente del esófago. Aunque clásicamente, el tratamiento de este tipo de tumores ha consistido en la enucleación por medio de una laparotomía o toracotomía, el auge de las técnicas endoscópicas y mínimamente invasivas ha revolucionado totalmente el manejo terapéutico de este tipo de tumores. Material y métodos: realizamos un estudio retrospectivo de todos los leiomiomas esofágicos intervenidos en el Hospital Universitario Ramón y Cajal entre el 1 de enero de 1986 y el 31 de diciembre de 2014, analizando características demográficas de los pacientes, sintomatología, localización tumoral, pruebas diagnósticas, datos quirúrgicos, complicaciones y estancia hospitalaria. Resultados: encontramos un total de 13 pacientes, siendo 8 varones y 5 mujeres, con una edad media de 53,62 años (rango 35-70 años). El tratamiento quirúrgico fue en todos los casos una enucleación. En 8 casos (61,54%) se realizó un abordaje torácico (4 toracotomías y 4 toracoscopias) y en 5 casos (38,56%) el abordaje fue abdominal (3 laparotomías y 2 laparoscopias). La enucleación se llevó a cabo a través de un abordaje mínimamente invasivo en 6 pacientes (46,15%). No hubo ningún caso de resección puramente endoscópica. La media de duración de la cirugía fue de 174,38 minutos (rango 70-270 minutos) y la mediana de estancia hospitalaria de 6,5 días (rango 2-27 días). No se describió ningún caso de mortalidad ni complicación intraoperatoria, aunque un paciente presentó importante dolor en hemitórax derecho que requirió manejo y seguimiento por la unidad del dolor. Con un seguimiento medio de 165,57 meses (mediana 170; rango 29-336 meses), no se han observado recidivas. Conclusiones: la enucleación constituye el tratamiento de elección en la mayor parte de los leiomiomas esofágicos. En nuestra experiencia, la duración de la cirugía es mayor tras cirugía mínimamente invasiva (CMI) que tras cirugía abierta (CA), sin embargo, la estancia media hospitalaria es menor. Paradójicamente, en valores absolutos, las complicaciones relacionadas con el dolor de la herida quirúrgica (neuralgia torácica) son mayores en el grupo de CMI. Sin embargo, ninguno de los resultados obtenidos en el trabajo es estadísticamente significativo, seguramente debidos al escaso tamaño muestral (AU)

Introduction: Leiomyomas are the most common benign tumors of the esophagus. Although classically surgical enucleation through thoracotomy or laparotomy has been widely accepted as treatment of choice, development of endoscopic and minimally invasive procedures has completely changed the surgical management of these tumors. Material and methods: We performed a retrospective review of all esophageal leiomyoma operated at Hospital Universitario Ramón y Cajal (Madrid, Spain) between January 1986 and December 2014, analyzing patients’ demographic data, symptomatology, tumor size and location, diagnostic tests, surgical data, complications and postoperative stay. Results: Thirteen patients were found within that period, 8 men and 5 women, with a mean age of 53.62 years (range 35-70 years). Surgical enucleation was achieved in all patients. In 8 cases (61.54%) a thoracic approach was performed (4 thoracotomies and 4 thoracoscopies), and in 5 cases (38.56%) an abdominal approach was performed (3 laparotomies and 2 laparoscopies); enucleation was carried out through a minimally invasive approach in 6 patients (46.15%). There were no cases of endoscopic resection alone. Surgery mean length was 174.38 minutes (range 70-270 minutes) and median postoperative stay was 6.5 days (range 2-27 days). There was neither mortality nor cases of intraoperative complications were described. No postoperative major complications were reported; however one patient presented important pain in his right hemithorax that required management and long term follow-up by the Pain Management Unit. With a mean follow-up of 165.57 months (median 170; range 29-336 months) no recurrences were reported. Conclusion: Enucleation is the treatment of choice for the majority of esophageal leiomyomas. In our experience, duration of the surgical procedure through minimally invasive approach was longer than surgery through open approach; however, postoperative stay was shorter in the first group. Paradoxically, incision pain after surgery (thoracic neuralgia) was found to be higher in the minimally invasive approach group. Nevertheless, none of the results obtained in the study reached statistical significance, probably due to the small simple size (AU)

Primary meningeal sarcoma with leiomyoblastic differentiation complicating pregnancy.

Intracranial malignant tumors during pregnancy are rare. Primary meningeal sarcoma with leiomyoblastic differentiation during pregnancy has not been reported. We present the case of a 25-year-old woman in the third trimester of pregnancy with a large intracranial tumor destructing the parietal calvaria and invasion of soft tissues. Histological examination revealed primary meningeal sarcoma with leiomyoblastic differentiation. A gross macroscopical resection of the tumor with removal of the infiltrated parietal calvaria was performed after delivery of a healthy baby through caesarean section. A mass on the head rapidly enlarging during pregnancy should be considered for a malignancy of intracranial origin. Early radiological exams as well as tumor resection followed by staging and multimodality treatment should be urgently performed.

Massive intraperitoneal hemorrhage caused by a giant exogastric leiomyoblastoma.

Leiomyoblastomas account for a small percentage of smooth muscle tumors of the stomach. Intraperitoneal bleeding is an unusual and unexpected presenting sign. We herein present a 43-year-old woman who was urgently operated on due to signs of collapse. A large hemorrhagic mass measuring 25 x 18 x 15 cm was found arising from the greater curvature of the stomach. A histologic examination demonstrated rounded and spindle cells, and rare mitoses were also seen. Although the number of mitoses was small, the lesion was nevertheless felt to be consistent with malignant leiomyoblastoma because of its large size. Three years later the patient is doing well with no evidence of tumor recurrence. We conclude that intraperitoneal bleeding due to leiomyoblastoma of the gastrointestinal tract is an extremely rare phenomenon and has been described only in a few reports, and only one other previous case presented with signs of collapse.

Hemoperitoneum due to a ruptured gastric stromal tumor.

BACKGROUND/AIMS: Gastrointestinal stromal tumors form a group of uncommon neoplasms originated from pluripotential mesenchymal cells. Many patients are asymptomatic and the tumor is discovered during an abdominal operation. Massive intraperitoneal bleeding is an exceptional complication associated with high mortality rates. Our aim is to report a case of a gastric stromal tumor in an 83-year-old patient presenting with intraperitoneal hemorrhage and hypovolemic shock, successfully operated. METHODS: Emergency laparotomy showed a hemoperitoneum caused by rupture of a large exogastric tumor attached to the greater curvature. Total gastrectomy and esophagojejunostomy was performed. RESULTS: Histological examination revealed proliferation of spindle-shaped cells but immunocytochemistry failed to identify specific markers of smooth muscle and neural cells. Diagnosis of a gastric stromal tumor was made. Postoperative evolution was uncomplicated. CONCLUSION: Gastric stromal tumor is a relatively rare neoplasm of mesenchymal origin whose nature and prognosis is unclear.

[Gastric stromal tumors. Results of a multicenter study. French Associations of Surgery Research]. / Tumeurs gastriques conjonctives. Résultats d'une étude multicentrique. Associations Françaises de Recherche en Chirurgie.

AIM OF THE STUDY: Gastric stromal tumours are not perfectly known. The aim of this retrospective multicenter study (29 centers) was to improve knowledge of these tumours. PATIENTS AND METHODS: From 1986 to 1994, 159 patients were operated on for leiomyomas (50), leiomyosarcomas (24), malignant/benign schwann cell tumours (10/29), automatic nerve tumours (4), leiomyoblastomas (28), spindle cell tumours (14). The mean duration of follow-up was 5 years. Presenting symptoms, diagnostic procedures, operative and pathological findings, evolution (recurrence, death) were recorded for each patient. RESULTS: Gastrointestinal bleeding and epigastric pain were the most common presenting symptoms (54% and 50% of patients, respectively). Endosonography was the most sensitive examination (97%). Malignant tumours size was greater than benign tumours size (12.6 cm versus 5.2 cm). Extension to contiguous organs or metastases were frequent (33% and 26% of patients, respectively). In 16 patients, pathological examination could not differentiate between malignant and benign tumour. Seven patients who had been operated on for a benign tumour (6%) developed a local (n = 4) or a metastatic (n = 3) recurrence. The 5-year survival rate was 40% for leiomyosarcomas, 28% for schwannosarcomas and 90% for malignant leiomyoblastomas. CONCLUSION: The main feature of stromal gastric tumours is the frequent difficulty to differentiate between malignant and benign tumours. The prognosis of malignant tumours depends on pathological types. The prognosis of benign tumours is uncertain since recurrences may develop.

Angiomiolipoma epiteloide renal / Epithelioid renal angiomyolipoma

Objetivo: Poner de manifiesto la existencia de una variedad de angiomiolipoma, denominada epitelioide, con propiedades diferentes tanto histológicas como clínicas. Método: Se presenta el caso de una mujer de 17 años, afecta de enfermedad de Bourneville, a la que en un control tomográfico renal se descubre la existencia de una masa sólida sugestiva de carcinoma, adyacente a otras de menor tamaño identificadas como angiomiolipomas. Resultados: Tras realizar varias tumorectomías, la sospechosa de carcinoma, de 4 cm de tamaño se diagnostica de angiomiolipoma epitelioide, con corroboración inmunohistoquímica mediante positividad para HMB45, y negatividad a vimentina y queratina. Conclusiones: A pesar de la posibilidad de coexistencia de adenocarcinoma y angiomiolipomas, no hay que descartar la existencia de la variedad epitelioide, sobre todo en pacientes con facomatosis. Los criterios quirúrgicos serán los mismos que para el resto de masas renales. Sin embargo, los criterios de seguimiento deberán ser más estrictos dada la posible evolución tórpida en cuanto a diseminación de esta infrecuente variedad de angiomilipoma (AU)

Objective: To issue the existence of a variety of angiomyolipoma, named epithelioid, with different histological and clinic properties. Methods: We report the case of a 17-year-old female, with Bourneville’s disease, who was discovered to have a solid renal mass suggestive of carcinoma in a control CT scan, adjacent to other smaller masses identified as angiomyolipomas. Results: After several tumorectomies, the suspicious mass, 4 cm in size, was diagnosed as epithelioid angiomyolipoma, with immunohistochemical confirmation of capacity for HMB45, and negative vimentin and keratin. Conclusions: Despite the possibility of coexistence of adenocarcinoma and angiomyolipoma, the existence of an epithelioid variety cannot be discarded, mainly in patients with phakomatosis. The indications for surgery are the same than for the rest of renal masses. Nevertheless, follow-up criteria must be stricter due to the possibility of torpid outcome in terms of dissemination of this infrequent variety of angiomyolipoma (AU)

Leiomioblastoma maligno de intestino delgado como causa de hemorragia digestiva baja grave / Malignant leiomyoblastoma of the small bowel due to acute lower gastrointestinal bleeding

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Tumor estromal gástrico maligno de crecimiento omental simulando un angioma / Gastrointestinal stromal tumor with omental enlargement mimicking an angioma

Los tumores del estroma gastrointestinal son raros y difieren histológicamente, inmunohistoquímicamente y genéticamente de los típicos leiomiomas, leiomiosarcomas y leiomioblastoma. En el caso que se presenta la evaluación radiológica mediante tomografía computarizada y resonancia magnética no pudo precisar su localización exacta de origen (tumor gástrico con crecimiento principal en el epiplón menor) ni detectar las metástasis a distancia (implantes peritoneales y mesentéricos). Su comportamiento radiológico era de angioma exofítico hepático o epiploico. Estas características se debieron al crecimiento en el epiplón y al importante componente angiomatoso presente en su constitución histológica. El tamaño tumoral y los implantes metastásicos lo clasificaron como maligno. El conocimiento de este tipo de tumores es importante para sospecharlos con las pruebas diagnósticas y planificar correctamente el tratamiento tras obtener muestras histológicas (AU)