Nomogram predicting overall survival after surgical resection for retroperitoneal leiomyosarcoma patients.

Background: Surgery is the best way to cure the retroperitoneal leiomyosarcoma (RLMS), and there is currently no prediction model on RLMS after surgical resection. The objective of this study was to develop a nomogram to predict the overall survival (OS) of patients with RLMS after surgical resection. Methods: Patients who underwent surgical resection from September 2010 to December 2020 were included. The nomogram was constructed based on the COX regression model, and the discrimination was assessed using the concordance index. The predicted OS and actual OS were evaluated with the assistance of calibration plots. Results: 118 patients were included. The median OS for all patients was 47.8 (95% confidence interval (CI), 35.9-59.7) months. Most tumor were completely resected (n=106, 89.8%). The proportions of French National Federation of Comprehensive Cancer Centres (FNCLCC) classification were equal as grade 1, grade 2, and grade 3 (31.4%, 30.5%, and 38.1%, respectively). The tumor diameter of 73.7% (n=85) patients was greater than 5 cm, the lesions of 23.7% (n=28) were multifocal, and 55.1% (n=65) patients had more than one organ resected. The OS nomogram was constructed based on the number of resected organs, tumor diameter, FNCLCC grade, and multifocal lesions. The concordance index of the nomogram was 0.779 (95% CI, 0.659-0.898), the predicted OS and actual OS were in good fitness in calibration curves. Conclusion: The nomogram prediction model established in this study is helpful for postoperative consultation and the selection of patients for clinical trial enrollment.

Treatment of vena cava inferior leiomyosarcoma with cavotomy and right renal vein neoimplantation / Vena cava inferior leiomyosarcoma kezelése cavotomiával és a jobb vena renalis neoimplantatiójával

Leiomyosacroma of the inferior vena cava is an extremely rare malignancy originating from the tunica media of the venous wall. Its symptoms and radiomorphological signs do not always lead to an accurate diagnosis. Histological sampling can be dangerous due to its location. Therefore the diagnosis is often a challenge for clinicians. Its treatment is primarily surgical, supplemented by radiotherapy and chemotherapy applied together or in monotherapy. In our case, an asymptomatic 74-year-old female patient was diagnosed with a tumor of the inferior caval vein located just above the right renal vein and involving the right adrenal gland. As serum cortisol, epinephrine, norepinephrine, adrenocorticotropic hormone (ACTH), total and free testosterone, dehydroepiandrosterone sulfate (DHEA-S), sex hormone binding globulin (SHBG) was at normal level, the tumor showed no hormone secretion. Primary surgical resection was planned. Preoperative biopsy was not performed due to its high risk of complications. During operation, tumor resection was performed by resection of the venous wall, removal of the right adrenal gland and neoimplantation of the right renal vein. For better exposure, cholecystectomy was also performed at the beginning of surgery. There were no postoperative complications. The patient received adjuvant radiation therapy. Thanks to the early diagnosis of the tumor and the complete resection, we significantly increased the patient's chances of total recovery. With the radiotherapy performed, we reduced the likelihood of tumor recurrence.

[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.

Challenging diagnostic and therapeutic modalities for leiomyosarcoma of inferior vena cava.

Leiomyosarcoma of the inferior vena cava (IVCL) is a rare malignant tumour originating from the smooth muscle cells of the media with intra- or extra-luminal growth. The type of the lesion is further divided into three levels in relation to hepatic and renal veins respectively. The aim of this review was to evaluate the results of surgical treatment of IVCL with special reference to the extent of its histological spread and to analyse the recent literature in order to provide an update on the current concepts of diagnostic and therapeutic management of this entity. IVCL's patients may present with non-specific complaints such as dyspnoea, malaise, weight loss, nausea, vomiting, fever and abdominal pain. Haematogenous metastasis is frequent. At a later stage, IVCL may also spread through lymphatic. Multiple diagnostic imaging techniques have been proposed for accurate preoperative diagnosis, including Doppler ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI), individually or in combination with cavography echocardiography or CT-guided biopsy. Despite recent research on the therapeutic strategies against IVCL, surgical resection appears the only potentially curative approach. Unfortunately, a mere minority of patients is eligible to undergo surgical intervention. In addition, surgical removal of IVCL does not necessarily guarantee patient's long-term survival. Alternative therapies, such as radio- and chemo-therapy often proved insufficient. Debate continues regarding the optimal management of the IVC after tumour resection, with primary repair, ligation and IVC reconstruction all have been utilized with varying success.

Synchronous adrenocortical neoplasms, paragangliomas, and pheochromocytomas: syndromic considerations regarding an unusual constellation of endocrine tumors.

The most common clinical syndromes presenting with paragangliomas and/or pheochromocytomas as their endocrine components are multiple endocrine neoplasia type 2, neurofibromatosis, Von Hippel-Lindau syndrome, Carney-Stratakis syndrome, Carney triad, and the recently described hereditary paraganglioma syndrome. Only Carney triad is known to also present with adrenocortical adenomas, currently representing the only described syndrome in which all 3 of the aforementioned tumors are found together. In most cases, prototypical lesions of the triad such as gastrointestinal stromal tumor and pulmonary chondromas are also seen. We present a case of a young woman with synchronous paragangliomas, adrenal/extra-adrenal cortical neoplasms, and pheochromocytoma without genetic mutations for multiple endocrine neoplasia 2, Von Hippel-Lindau syndrome, neurofibromatosis, and succinate dehydrogenase. We speculate that this represents a previously undescribed presentation of Carney triad and, at the very least, indicates the need for monitoring for the development of other tumors of the triad.

Survey among German gynecologists on the clinical management of patients with sarcomas of the uterus.

AIM: To gain more information about the knowledge of the clinical management of uterine sarcoma. MATERIALS AND METHODS: This survey was performed among members of the North-Eastern German Society of Gynecological Oncology (NOGGO) and the German Society of Psychosomatic Medicine in Gynecology and Obstetrics (DGPFG) on the treatment of uterine sarcomas. RESULTS: Altogether, 374 gynecologists took part. When asked about the surgical therapy of leiomyosarcoma, 64% indicated hysterectomy with bilateral adenectomy and lymph node dissection. Answers on the extent of lymphadenectomy in leiomyosarcoma differed widely. When asked about the preferred chemotherapy regimen for metastatic uterine sarcoma, more than 60% of all gynecologists would not apply any chemotherapy. Almost 40% recommended any kind of radiotherapy in this situation. CONCLUSION: There is a great uncertainty about the standard treatment of uterine sarcoma, even among specialists of gynecological oncology. It is time for organized efforts to improve the treatment of uterine sarcoma.

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.

Permanent iodine-125 interstitial planar seed brachytherapy for close or positive margins for thoracic malignancies.

PURPOSE: To assess toxicity and outcome following permanent iodine-125 seed implant as an adjunct to surgical resection in cases of advanced thoracic malignancy. METHODS AND MATERIALS: An institutional review board-approved retrospective review was performed. Fifty-nine patients were identified as having undergone thoracic brachytherapy seed implantation between September 1999 and December 2006. Data for patient demographics, tumor details, and morbidity and mortality were recorded. RESULTS: Fifty-nine patients received 64 implants. At a median follow-up of 17 months, 1-year and 2-year Kaplan-Meier rates of estimated overall survival were 94.1% and 82.0%, respectively. The 1-year and 2-year local control rates were 80.1% and 67.4%, respectively. The median time to develop local recurrence was 11 months. Grades 3 and 4 toxicity rates were 12% at 1 year. CONCLUSIONS: This review shows relatively low toxicity for interstitial planar seed implantation after thoracic surgical resection. The high local control results suggest that an incomplete oncologic surgery plus a brachytherapy implant for treating advanced thoracic malignancy merit further investigation.

[Bladder leiomyosarcoma. Partial cystectomy and adjuvant treatment]. / Leiomiosarcoma de vejiga: cistectomía parcial y tratamiento complementario.

OBJECTIVE: We report a new case of bladder leiomyosarcoma in an old patient. METHODS: We present the case of a 75-year-old man with bladder leiomyosarcoma treated by partial surgery followed by adjuvant treatment. RESULTS: Partial surgery of the primary tumor followed by concomitant chemoradiotherapy was the approach for this patient. Nowadays, patient is free of tumor and living without any problems. CONCLUSIONS: Bladder leiomyosarcoma is an uncommon tumor (only about 1% of all bladder cancers) treated basically with radical surgery. Nowadays, partial surgery is a usual approach in other tumors and there is a trend toward less aggressive surgery with preservation of function (such as head and neck cancer, bladder cancer).

Leiomiosarcoma de vejiga: cistectomía parcial y tratamiento complementario / Bladder leiomyosarcoma. Partial cystectomy and adjuvant treatment

OBJETIVO: realizar una revisón de las posibilidades terapéuticas del leiomiosarcoma vesical.MÉTODO: Presentamos el caso de un varón de 75 años con leiomiosarcoma vesical tratado mediante cirugía parcial y un tratamiento complementario tras la cirugía.RESULTADOS: El paciente fue sometido a cirugía parcial seguido de radioterapia locorregional así como quimioterapia complementaria en régimen ifosfamida-adriamicina.CONCLUSIONES: El leiomiosarcoma de vejiga es un tumor poco frecuente (únicamente 1% de todos los tumores vesicales) en el que la piedra angular del tratamiento es la cirugía. Hoy en día, las cirugías parciales son una alternative terapéutica en otros tumores, observando cada vez más un mayor número de cirugías conservadoras para conservar función(AU)

OBJECTIVE: We report a new case of bladder leiomyosarcoma in an old patient.METHODS: We present the case of a 75-year-old man with bladder leiomyosarcoma treated by partial surgery followed by adjuvant treatment.RESULTS: Partial surgery of the primary tumor followed by concomitant chemo-radiotherapy was the approach for this patient. Nowadays, patient is free of tumor and living without any problems.CONCLUSIONS: Bladder leiomyosarcoma is an uncom-mon tumor (only about 1% of all bladder cancers) treated ba-sically with radical surgery. Nowadays, partial surgery is a usual approach in other tumors and there is a trend toward less aggressive surgery with preservation of function (such as head and neck cancer, bladder cancer)(AU)

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)

Leiomiosarcoma de la vulva / Leiomyosarcoma of the vulva

Se presenta un caso de leiomiosarcoma de la vulva (LMS-V), tumor maligno de frecuencia excepcional; según la extensa revisión bibliográfica efectuada, el aquí recogido tendría el número 47 de los publicados. El seguimiento comprende más de 5 años y la evolución ha sido favorable. Se analizan la apariencia clínica de benignidad, los criterios histológicos para su caracterización y gradación histológicas y los tratamientos empleados, de los cuales el tratamiento quirúrgico primario con suficiente margen de seguridad parece ser el más eficaz para conseguir una evolución favorable (AU)

[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.

Completely aborted uterine leiomyosarcoma in the puerperium.

A patient passed a large tissue mass at home on postpartum day 1. Pathologic examination demonstrated leiomyosarcoma. A total abdominal hysterectomy was performed, which demonstrated residual disease. All tissue passed in the puerperium should be evaluated to ensure that significant pathologic conditions are not overlooked. Conservative management of leiomyosarcomas is controversial.

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)

Rectal leiomyosarcoma diagnosed by endoscopic ultrasonography.

A 67 year-old man was admitted to the Tainan Municipal Hospital due to a protruding mass, usually noted during defecation. Digital examination revealed a single, smooth, large mass over the rectum, occupying almost the entire lumen. Colonoscopy, barium enema, and computed tomography (CT) demonstrated a submucosal tumor of the rectum. Endoscopic ultrasound (EUS) study showed that the tumor originated from the muscle layer. Based on the size, margin and echogenicity of the mass, a malignant neoplasm, probably leiomyosarcoma, was diagnosed. Post-operative histologic examination confirmed that the resected tumor was leiomyosarcoma. Existing ancillary procedures like colonoscopy, abdominal CT, magnetic resonance image (MRI), and barium enema are neither reliable nor accurate in locating which layer the lesion originates. Colonoscopic biopsy is disappointing since submucosal tumor is usually inaccessible. EUS study can provide us with a more distinct image with regards to tumor origin, size, margin and echogenicity. This report emphasizes the important role of EUS in the pre-operative diagnosis of submucosal tumors of the rectum. Furthermore, this tool can aid the surgeons whether wide excision or an abdomino-perineal resection should be performed.

[A case of liver metastases from leiomyosarcoma in the chest wall which was made resectable by chemoembolization].

We here report a recently experienced case in which TAE and intra-arterial infusion chemotherapy for treatment of liver metastases of leiomyosarcoma in the chest wall caused a shrinking of the metastasized focus, thus facilitating liver resection. Patient; a 38-year-old man Present History and Courses; Resection of leiomyosarcoma in his chest wall was done in May, 1994. However, a local recurrence was noted in September, 1995, and the tumor was removed. Then, he received systemic chemotherapy with CDDP (100 mg) and ADM (45 mg). Abdominal CT and ultrasonic examinations made in February, 1996 revealed liver metastases at S2,5,8. Angiography detected densely stained images of tumors at a number of sites along with S2,5,8. Since these were thought unresectable, TAE therapy with EPIR (30 mg) and lipiodol (4 ml) was attempted 3 times. Then, a reservoir for intra-hepatic arterial infusion was implanted in April, 1996 and EPIR at a dose of 30 mg (150 mg in total) was given through arterial infusion, resulting in tumor disappearance at S5,8 but further growth of the tumor metastasizes at S2. Therefore, a resection of the left lateral segment of liver was done August 23. Though he was discharged in October, metastasis was found in the thoracic spine in December. Thus, he underwent resection of the vertebral arch including the tumor.

[Retroperitoneal tumors: personal experience]. / I tumori retroperitoneali: esperienza personale.

The Authors experience in five retroperitoneal tumors with symptoms and histopathological characteristics not different from those described in the Literature, is here reported. Topographic findings, classification, and incidence of retroperitoneal tumors as referred by various Authors are discussed. Two aspects are particularly pointed out: the histological type and the surgical strategy for their removal. Concerning the first aspect, the Authors underline that benign lesion may have an optimal outcome and a long survival, unlike the malignant ones, which have always unfavourable prognosis, despite adjuvant and complementary therapy (radiotherapy, chemotherapy). As for the second aspect, surgery is the treatment of choice even when other organs different from those exclusively retroperitoneal, may be involved in the demolition.