Leiomyosarcoma of the small intestine presenting as abdominal myofascial pain syndrome (AMPS): case report.

OBJECTIVE: To highlight an extremely unusual presentation of an aggressive, rare small bowel malignancy presenting as abdominal myofascial pain syndrome. CASE PRESENTATION: The report is presented from a tertiary pain medicine unit at a university teaching hospital. A female patient presenting with chronic abdominal pain was initially diagnosed as abdominal myofascial pain syndrome. The report details the possible facilitation of the diagnosis of a rare, highly aggressive small bowel tumour by interventional treatment for abdominal myofascial pain syndrome. CONCLUSION: This case highlights a rare and aggressive malignancy of the small intestine presenting clinically as abdominal myofascial pain syndrome.

[Carney triad]. / Carneyho trias.

Carney triad is a synchronous or metachronous association of gastric gastrointestinal stromal tumors (GIST), pulmonary chondroma and extra-adrenal paraganglioma. The majority of patients have only one or two components of the triad, all three tumors being found in only about 2% of the patients at the time of the first diagnosis. The most common combination is gastric and pulmonary tumors. We report a case of Carney triad which was diagnosed at Masaryk Memorial Cancer Institute. A 57-year-old female patient with a history of gastric resection for leiomyosarcoma at the age of 14 and with an unclear pulmonary lesion evident on chest X-ray since as early as 2003. She was referred to our Clinic of Comprehensive Cancer Care after being diagnosed with unspecified tumors of the stomach, the left retroperitoneum and two liver metastases. Biopsy of the retroperitoneal mass was performed and histological examination showed pheochromocytoma. The patient underwent resection of the retroperitoneal tumor and wedge resection of the gastric tumor, left hemihepatectomy and left adrenalectomy (in two separate operations). The excised gastric tumor was a gastrointestinal stromal tumor (GIST) with a low risk of malignancy. Analysis of a liver specimen, however, showed two GIST metastases. No pathology was found in the left adrenal gland and the retroperitoneal tumor was positive for chromogranin A. Paraganglioma was thus diagnosed. Subsequently, mutational analysis of genes coding for succinate dehydrogenase subunits B, C and D (SDHB, SDHC, SDHD) and analysis of DNA methylation at the gene locus of SDHC was made. Carney triad was thus confirmed and the unclear pulmonary lesion could be described as benign chondroma. This report demonstrates the difficulty in distinguishing between Carney triad and Carney-Stratakis syndrome. Molecular information should improve the diagnosis of Carney triad.Key words: Carney triad - GIST pulmonary chondroma extraadrenal paragangliomaCarney-Stratakis syndrome.

[Molecular pathology of sarcomas. Update on the research group "Molecular Diagnosis of Sarcomas"]. / Molekularpathologie von Sarkomen. Erste Ergebnisse des Sarkomforschungsverbundes KoSar.

To establish precise diagnostic algorithms and standardised treatment of sarcomas in specialized centers, the interdisciplinary research group KoSar (sarcoma competence network) has been funded by German Cancer Aid. A sarcoma tissue repository and a diagnostic reference center have been set up, presently containing about 1000 accurately diagnosed sarcomas of different entities. Significant gene expression profiles for synovial sarcomas, leiomyosarcomas, myxoid liposarcomas and a small profile for myxofibrosarcomas as well as a new classification of angiosarcomas were defined. We systematically searched for activated signal transduction pathways in sarcoma cell lines and xenograft transplant models and candidate targets for molecular therapies were identified. Based on these results first clinical studies have been initiated by the German Interdisciplinary Sarcoma Study Group (GISG).

Diagnosis and management of primary pulmonary leiomyosarcoma.

Leiomyosarcomas are cancers of smooth muscle cells that can arise from any location but occur most often in the uterus, retroperitoneum, or intraabdominal region. Primary leiomyosarcomas of the lung are extremely rare and are often diagnosed as a mass on routine chest radiography. Metastasis is uncommon and typically occurs late in the disease process, indicating the importance of early detection. Surgical excision is generally curative, offering patients optimal prognoses. The authors describe a 56-year-old asymptomatic man who was found to have a pulmonary mass on routine chest radiography. Further testing revealed a primary pulmonary leiomyosarcoma. The tumor was excised and postoperative osteopathic manipulative treatment was applied to help facilitate patient recovery. A brief discussion of the typical clinical presentation, prevalence, and medical management of these tumors is included.

Leiomiosarcoma renal: 2 casos clínicos / Renal leiomyosarcoma

El leiomiosarcoma de origen renal es una patología rara y agresiva, más frecuente en mujeres y con gran tendencia a la recurrencia local y a distancia. Describimos dos casos clínicos de leiomiosarcoma renal, el primero correspondiente a una mujer de 46 años con gran lesión tumoral retroperitoneal derecha que fue sometida a nefrectomía radical y recibió tratamiento complementario con radioterapiaal lecho renal. El segundo caso corresponde a un hombre de 71 años con hallazgo de lesión tumoral renal izquierda cuyo tratamiento consistió en nefrectomía radical. El informe anatomopatológico fue compatible con leiomiosarcoma renal con alto grado de malignidad en ambos casos. El tratamiento de elección es la nefrectomía radical, con tratamiento adyuvante con quimioterapia y/o radioterapia. Se realiza un análisis de la literatura.

Renal leiomyosarcoma is a rare and aggressive disease, with a higher incidence on women and atendency for local and distant recurrence. We report two cases. A 46 year old female with aretroperitoneal lesion that underwent radical nephrectomy and complementary radiotherapy of renal fossa. A 71 year old man with a left renal tumor that underwent radical nephrectomy. Pathology informed a high grade leiomyosarcoma in both cases. Radical nephrectomy is the treatment of choice along with adjuvant chemo and radiotherapy Literature is reviewed.

Leiomiosarcoma de cava inferior. Hallazgo incidental / Leiomyosarcoma of the inferior vena cava. Incidental finding

Objetivo: El leiomiosarcoma de cava inferior es una entidad poco frecuente, clínicamente insidiosa o silente y detectable en muchas ocasiones únicamente mediante estudios de imagen. Se presenta un caso intervenido en nuestro servicio y se revisa la literatura al respecto. Método: Mujer de 58 años con una masa suprarrenal derecha de 6 cm evidenciando su origen en la pared de vena cava durante el acto quirúrgico. Se realiza exeresis completa de la tumoración, y posteriormente , radioterapia adyuvante sobre el lecho quirúrgico. Resultados: Tras más de 2 años de evolución, la paciente se encuentra libre de enfermedad. Conclusiones: Esta entidad presenta una escasa prevalencia, y en muchas ocasiones su hallazgo es incidental. La resección quirúrgica completa es la clave del tratamiento, aunque la probabilidad de recidiva local es elevada (AU)

Objective: Leiomyosarcoma of the inferior vena cava is a rare tumor, clinically silent which often remains undiagnosed for much longer. Imaging methods allow us to detect these entities. We report a single case and perform a bibliographic review. Methods: 58-year-old woman with a 6 cm adrenal mass, which during surgery was found to be a tumor from the wall of the vena cava. We performed complete removal of the mass. Radiotherapy of the surgical area was applied within three months following surgery. Results: Two years later, there is no evidence of disease recurrence. Conclusion: This is a rare entity, with low prevalence. Complete surgical excision is the gold standard for treatment. Local recurrence is a common finding during follow up (AU)

Aggressive recurrence after radiofrequency ablation of liver neoplasms.

Radiofrequency ablation is considered safe for inoperable liver neoplasms; with small lesions the rate of success is very high, the local recurrence is marginal and generally suitable for a retreatment. We have little information about the possibility of rapid regrowth of the tumor after a response judged as complete. We present four patients, affected by primary (3 patients) and metastatic (1 patient) uninodular cancer. All the lesions were small, superficial and well suited for surgery, but were treated by radiofrequency ablation elsewhere. The early instrumental evaluations stated a complete result in all the patients. Cancer regrowth was diagnosed at 3, 4, 6 and 12 months after radiofrequency ablation, always starting from the treated lesion. In case 1 the whole right lobe was involved together with a controlateral multinodular recurrence; cases 2 and 3 presented an extensive liver and parietal wall involvement; while in the fourth patient a diffuse biliary colonization was observed. Only 1 patient was suitable for surgery; the others died 6, 2 and 4 months, respectively, after recurrence. Recurrence after radiofrequency ablation may show an aggressive evolution precluding any possibility of cure. Radiofrequency ablation must not be considered a suitable alternative to surgery in patients with a low surgical risk.

A parathyroid hormone-related protein-secreting metastatic epithelioid leiomyosarcoma. A case report and review of the literature.

A diagnosis of parathyroid hormone-related protein (PTH-rP)-secreting metastatic uterine epithelioid leiomyosarcoma was made in a 61-year-old woman with humoral hypercalcemia of malignancy. A primary uterine tumor had been removed 10 years previously, which had been associated with a short history of hypercalcemia. The original uterine tumor was diagnosed as a smooth muscle tumor of uncertain malignant potential. To the best of our knowledge, this is the first reported case of a PTH-rP-secreting uterine leiomyosarcoma. We demonstrate the dramatic changes in serum calcium, phosphorus, PTH, and PTH-rP levels after tumor resection. Extensive biochemical analysis and detailed immunohistochemical and ultrastructural characterization demonstrate several features of this tumor.

[A case of stromal gastric tumor-uncommon clinical and pathological entity]. / Un caz de tumora stromala gastrica--raritate clinica si histopatologica.

Stromal gastric tumors represents an uncommon entity with difficult diagnosis, both preoperative and pathological. We present the case of a patient admitted for abdominal pain, with palpable tumor in epigastrium and right hypochondrium; abdominal echography, barium enema and abdominal CT-scan were not able to precise the involved organ. Operative exploration found at the level of horizontal portion of the stomach a solid tumor, which was resected, with good subsequent evolution. Pathologic diagnosis was stromal gastric tumor. We present latest news concerning diagnosis, prognostic and treatment for this type of tumor.

Metástasis hepáticas secundarias a leiomiosarcoma de recto. Presentación de un caso / Liver metastases secondary to rectal leiomyosarcoma. A case report

Se presenta un caso de metástasis hepáticas en una paciente afectada por un leiomiosarcoma de localización rectal. Se incide en la rareza de este tipo de tumores, así como en su agresividad y posibilidades terapéuticas (AU)

Unusual cutaneous malignancies.

The most commonly occurring cutaneous malignancies are basal cell and squamous cell carcinoma. There are, however, other rare malignancies that are encountered and should be included in the differential, in which both the clinical and the histological diagnosis may be difficult. Here, the clinical and histological features, management, and prognostic factors of merkel cell carcinoma, microcystic adnexal carcinoma, leiomyosarcoma, dermatofibrosarcoma protuberans, and angiosarcoma are reviewed.

[The value of dynamic MRT in the perfusion evaluation of pelvic tumors under hyperthermia]. / Uber die Wertigkeit der dynamischen MRT in der Perfusionsbeurteilung von Beckentumoren unter Hyperthermie.

For the purpose of assessing perfusion of deep seated pelvic tumours 22 dynamic MR studies were carried out and correlated with relevant CT studies as well as with the intratumoural distribution of temperature under hyperthermia in different areas. Using changes of signal intensity, dynamic MR could thus distinguish between well and poorly perfused tumours, besides supplying reliable information on inhomogeneities in tumour perfusion. Altogether, a correlation of 0.92 (p < 0.001) was found between MR and CT and 0.72 (p < 0.001) between MR and the steady state determination of intratumoural temperature. Thus, dynamic MR provides prognostic information concerning therapeutic temperatures (> = 42 degrees C) in tumours and therefore their response to hyperthermia.

Abscessed leiomyosarcoma of the ileum.

A-49-year-old man was admitted because of a lower abdominal mass. During the five days prior to admission, he had noted fever and lower abdominal pain. Palpation of the abdomen revealed a tender mass, 10 cm in diameter, in the suprapubic region. An x-ray revealed an irregular collection of gas, 9 cm in diameter, in the pelvic cavity, which appeared as a mirror image when the patient was upright. Based on the physical finding and the results of a barium enema, abdominal CT, MRI, and small intestine imaging, a diagnosis of leiomyosarcoma of the ileum was made. Excision of the tumor and part of the small intestine was performed. A saccular tumor, 11 x 6 x 5 cm, was found 1.0 m from the cecum and growing out of the wall of the ileum. A large amount of pus was found inside the cavity. The pathological diagnosis was leiomyosarcoma.

Integrated magnetic resonance imaging and phosphorus spectroscopy of soft tissue tumors.

Eighteen patients with soft tissue masses underwent integrated magnetic resonance imaging (MRI) and phosphorus spectroscopy (31P-MRS) to evaluate benign and malignant tumor morphology and metabolism. Spectra from soft tissue tumors had a significantly higher proportion of phosphate in the low-energy portion of the 31P spectrum (P less than 0.001) with a concomitant decrease in phosphocreatine (P less than 0.01) compared with 31P spectra from normal muscle. Malignant tumors had a mean pH of 7.35 +/- 0.13 which was greater than that of muscle tissue with a mean pH of 7.08 +/- 0.07 (P less than 0.001). All tumors had greater relative levels of phosphomonoesters, inorganic phosphate, and phosphodiesters compared with those in muscle tissue but considerable variability among tumors was noted due to tumor size, extent of tumor necrosis, and muscle contamination. Integrated MRI/MRS studies are necessary to provide exact localization of the tumor and a more correct interpretation of the 31P-MRS data.

[Leiomyosarcoma of the esophagus. Apropos of a case with a review of the literature]. / Léiomyosarcome de l'oesophage. A propos d'un cas avec revue de la littérature.

The authors report a new case of leiomyosarcoma of the oesophagus, and a review of the literature since 1960 (during this period almost every case has been resected). Four macroscopic aspects are observed: polypoid, mediastinal, intra-mural and infiltrating. The accurate diagnosis is difficult and was established before the operation only in half cases. It is based on barium swallow examination and esophagoscopy, that reveal different appearances depending on the anatomical type, and on the biopsy. The interpretation of the biopsy may be difficult because of the superficial nature of the sampling, frequently taking only mucosa or necrotic tissue. It is necessary to remove the necrotic tissue to discover the tumoral tissue, or to perform a wide diathermic snare biopsy. The diagnosis of malignancy is mainly based on the mitosis count, and interpretation of the peroperative pathologic examination is difficult. The surgeon may have to decide on an esophageal resection, according to the gross appearance. Esophagectomy is the most logical treatment; adjuvant radiation therapy could have some efficiency.