Leiomyosarcomas affecting main vessels in the lower extremities. / Leiomiosarcomas de grandes vasos en las extremidades inferiores.

OBJECTIVE: To evaluate the results of bloc resection and vascular reconstruction of leiomyosarcomas with involvement of main vessels in the lower extremities. MATERIAL AND METHODS: From January 1983 to December 2016, 42 patients with leiomyosarcomas were diagnosed. Six of these leiomyosarcomas affected main vessels of the lower extremities (called vascular). Epidemiological data, imaging studies, surgery performed, adjuvant treatments, complications, as well as recurrences and mortality were retrospectively recorded. RESULTS: All the patients were affected by high-grade leiomyosarcomas (ii-iii FNCLCC classification), with a larger tumour average diameter of 9.1cm(6-15) and a mean follow-up of 23 months (7-36). The average age was 64 years (29-84). The first symptom was a palpable tumour in 4 of them. The other 2 cases debuted with thromboembolic phenomena. In 5 cases the origin was the femoral vessels, while one case was at the popliteal level. Although all cases preserved the limb, in 3 cases (50%) they presented pulmonary dissemination,2 cases (33%) hepatic dissemination and one case had local recurrence. Two cases died at the end of the study and there was one case of loss to follow-up. DISCUSSION AND CONCLUSIONS: Vascular leiomyosarcomas are highly aggressive tumours with a low survival rate at 5 years. In our study, 50% of the patients remain in complete remission with a mean follow-up of 23 months. Their onset frequently associates the presence of tumour mass with thrombotic phenomena (33% of our cases). Tumour resection surgery usually compromises the main vascular structures, which implies resection and vascular reconstructive techniques to salvage the limb.

Trombosis venosa ovárica posparto: una causa inusual de fiebre puerperal / Postpartum ovarian venous thrombosis: An unusual cause of puerperal fever

La trombosis de la vena ovárica posparto es una complicación rara e impredecible y que requiere un alto índice de sospecha clínica, cuyo diagnóstico y manejo precoces son esenciales, requiriendo un abordaje multidisciplinar, para evitar cirugías innecesarias y posibles complicaciones graves. Su presentación clínica habitual suele ser fiebre persistente y dolor abdominal, generalmente en la primera semana posparto. Revisamos 3 casos clínicos representativos, diagnosticados en nuestro centro entre 2014 y 2019, abordando los factores predisponentes, diagnóstico, manejo y evolución de estas pacientes.(AU)

Postpartum ovarian vein thrombosis is a rare and unpredictable complication that requires a high index of clinical suspicion. Its early diagnosis and management, with a multidisciplinary approach, are essential in order to avoid unnecessary surgeries and possible serious complications. Its usual clinical presentation is often persistent fever and abdominal pain, generally in the first week postpartum. A report is presented on three representative cases that were diagnosed in our centre between 2014 and 2019, presenting predisposing factors, diagnosis, management, and outcome of these patients.(AU)

Germline RET 634 mutation positive MEN 2A-related C-cell hyperplasias have genetic features consistent with intraepithelial neoplasia.

C-cell hyperplasias are normally multifocal in multiple endocrine neoplasia type 2A. We compared clonality, microsatellite pattern of tumor suppressor genes, and cellular kinetics of C-cell hyperplasia foci in each thyroid lobe. We selected 11 females from multiple endocrine neoplasia type 2A kindred treated with thyroidectomy due to hypercalcitoninemia. C-cell hyperplasia foci were microdissected for DNA extraction to analyze the methylation pattern of androgen receptor alleles and microsatellite regions (TP53, RB1, WT1, and NF1). Consecutive sections were selected for MIB-1, pRB1, p53, Mdm-2, and p21WAF1 immunostaining, DNA content analysis, and in situ end labeling. Appropriate tissue controls were run. Only two patients had medullary thyroid carcinoma foci. Nine informative C-cell hyperplasia patients showed germline point mutation in RET, eight of them with the same androgen receptor allele preferentially methylated in both lobes. C-cell hyperplasia foci showed heterogeneous DNA deletions revealed by loss of heterozygosity of TP53 (12 of 20), RB1 (6 of 14), and WT1 (4 of 20) and hypodiploid G0/G1 cells (14 of 20), low cellular turnover (MIB-1 index 4.5%, in situ end labeling index 0.03%), and significantly high nuclear area to DNA index ratio. MEN 2A (germline point mutation in RET codon 634) C-cell hyperplasias are monoclonal and genetically heterogeneous and show down-regulated apoptosis, findings consistent with an intraepithelial neoplasia. Concordant X-chromosome inactivation and interstitial gene deletions suggest clone expansions of precursors occurring at a point in embryonic development before divergence of each thyroid lobe and may represent a paradigm for other germline mutations.

Contribution of the microvessel network to the clonal and kinetic profiles of adrenal cortical proliferative lesions.

Monoclonal adrenocortical lesions have been characterized by an inverse correlation between proliferation and apoptosis, and polyclonal lesions show a direct correlation. Their relationship with the vascular pattern remains unknown in adrenocortical nodular hyperplasias (ACNHs), adenomas (ACAs), and carcinomas (ACCs). We studied 20 ACNHs, 25 ACAs, and 10 ACCs (World Health Organization classification criteria) from 55 women. The analysis included X-chromosome inactivation assay (on microdissected samples), slide and flow cytometry, and in situ end labeling. Endothelial cells were stained with anti-CD31, and the blood vessel area and density were quantified by image analysis in the same areas. Appropriate tissue controls were run in every case. Regression analyses between kinetic and vascular features were performed in both polyclonal and monoclonal lesions. Polyclonal patterns were observed in 14 of 18 informative ACNHs and 3 of 22 informative ACAs, and monoclonal patterns were seen in 4 of 18 ACNHs, 19 of 22 ACAs, and 9 of 9 ACCs. A progressive increase in microvessel area was observed in the ACNH-ACA-ACC transition but was statistically significant between benign and malignant lesions only (191.36 +/- 168.32 v 958.07 +/- 1279.86 microm(2); P < .0001). In addition, case stratification by clonal pattern showed significant differences between polyclonal and monoclonal benign lesions; 6% of polyclonal and 57% of monoclonal lesions had microvessel area >186 microm(2) (P = .0000008). Monoclonal lesions showed parallel trends (but with opposite signs) for microvessel area and density in comparison with proliferation and apoptosis, whereas polyclonal lesions showed inverse trends. In conclusion, the kinetic advantage of monoclonal adrenal cortical lesions (increased proliferation, decreased apoptosis) is maintained by parallel increases in microvessel area and density.

Critical analysis of histologic criteria for grading atypical (dysplastic) melanocytic nevi.

Low concordance in grading atypical (dysplastic) melanocytic nevi (AMN) has been reported, and no systematic evaluation is available. We studied 123 AMN with architectural and cytologic atypia (40 associated with atypical-mole syndrome), classified according to standard criteria by 3 independent observers. Histologic variables included junctional and dermal symmetry, lateral extension, cohesion and migration of epidermal melanocytes, maturation, regression, nuclear features, nuclear grade, melanin, inflammatory infiltrate location, and fibroplasia. AMN (43 junctional and 80 compound) were graded mild (31), moderate (61), and severe (31). AMN-severe correlated with 3 or more nuclear abnormalities (especially pleomorphism, heterogeneous chromatin, and prominent nucleolus) and absence of regression, mixed junctional pattern, and suprabasilar melanocytes on top of lentiginous hyperplasia. AMN-severe diagnostic accuracy was 99.5% using these criteria, but only the absence of nuclear pleomorphism differentiated AMN-mild from AMN-moderate. No architectural features distinguishing AMN-mild from AMN-moderate were selected as significant by the discriminant analysis. AMN from atypical-mole syndrome revealed subtle architectural differences, but none were statistically significant in the discriminant analysis. Histologic criteria can reliably distinguish AMN-severe but fail to differentiate AMN-mild from AMN-moderate. AMN from atypical-mole syndrome cannot be diagnosed using pathologic criteria alone.

Molecular and kinetic features of transitional cell carcinomas of the bladder: biological and clinical implications.

Molecular and kinetic analyses have contributed to our understanding of the biology of transitional cell carcinomas (TCC) of the bladder. The concordant pattern of X-chromosome inactivation of multiple TCCs appearing at different times and at different sites and concordant genetic abnormalities in a subset of muscle-invasive TCC strongly support a monoclonal origin and a homogeneous tumor cell selection throughout the neoplasm. However, topographic intratumor heterogeneity results from the accumulation of genetic lesions in tumor suppressor genes, predominantly neurofibromatosis (NF)-1-defective in the superficial compartment and tumor protein p53 (TP53)-defective in the deep one, with lower proliferation and down-regulation of apoptosis in the latter. TCCs follow the general concept of multistep carcinogenesis and proceed through two distinct genetic pathways responsible for generating different TCC morphologies. These are the inactivation of cyclin-dependent kinase inhibitors (p15, p16, and p21WAF/CIP1) in low-grade TCC and early TP53-mediated abnormalities in high-grade TCC. TCC progression correlates with genetic instability and accumulation of collaborative genetic lesions mainly involving TP53, retinoblastoma (RB)-1, and growth factors. Distinctive genetic (low incidence of RB-1 and NF-1 abnormalities) and kinetic (slower cell turnover) profiles also correlate with a "single-file" infiltration pattern and poor survival in muscle-invasive TCCs. The underlying molecular changes of carcinoma in situ involve multiple and more extensive deletions (normally TP53-defective) than coexistent invasive TCC, suggesting an independent genetic evolution, while low-grade dysplasia is mainly polyclonal and shows a low rate of gene deletions.

PCR techniques for clonality assays.

Clonal overgrowths represent the hallmark of neoplastic proliferations, and their demonstration has been proved useful clinically for the diagnosis of malignant lymphomas based on the detection of specific and dominant immunoglobulin and/or T-cell receptor gene rearrangements. Nonrandom genetic alterations can also be used to test clonal expansions and the clonal evolution of neoplasms, especially analyzing hypervariable deoxyribonucleic acid (DNA) regions from patients heterozygous for a given marker. These tests rely basically on the demonstration of loss of heterozygosity (LOH) resulting from either hemizygosity (nonrandom interstitial DNA deletions) or homozygosity of mutant alleles observed in neoplasms. LOH analyses identify clonal expansions of a tumor cell population, and point to monoclonal proliferation when multiple and consistent LOH are demonstrated. Based on the methylation-related inactivation of one X chromosome in female subjects, X-linked markers (e.g., androgen receptor gene) will provide clonality information using LOH analyses after DNA digestion with methylation-sensitive restriction endonucleases. Therefore, both non-X-linked and X-linked analyses give complementary information, related and not related to the malignant transformation pathway respectively. Applied appropriately, these tools can establish the clonal evolution of tumor cell populations (tumor heterogeneity), identify early relapses, distinguish recurrent tumors from other metachronic neoplasms, and differentiate field transformation from metastatic tumor growths in synchronic and histologically identical neoplasms.

Early changes in the Schwann cells in experimental allergic neuritis.

Experimental allergic neuritis (EAN) was induced in guinea-pigs by intradermal injection of rabbit peripheral nerve emulsified in Freund's adjuvant. Both sciatic nerves were obtained between 12--24 hr after clinical symptoms were evident. Several fascicles from each nerve were isolated for histochemical studies with NADH-diaphorase (NADH) and acid phosphatase (AP) applied to teased nerve fibres. Small pieces were processed for electron microscopy, and a fascicle was teased after staining with osmium tetroxide. In isolated nerve fibres stained with histochemical techniques myelin lesions of segmental character were found closely related to inflammatory cells; Schwann cell cytoplasm in contact with mononuclear cells showing a heavy enzymatic activity (NADH and AP). Under polarized light, the underliying myelin showed focal loss of birefringence. Some electron-microscopic pictures suggested active myelin breakdown by mononuclear cells. The possibility of primary Schwann cell damage by mononuclear cells with subsequent demyelination is discussed.

Merkel cell (neuroendocrine) carcinoma of the vulva. A case report with immunohistochemical and ultrastructural findings and review of the literature.

A new case of primary Merkel cell carcinoma (MCC) of the vulva is reported and the literature reviewed for noting its clinical presentation, microscopic, immunohistochemical and ultrastructural features, as well as for establishing the role of immunohistochemistry in the ultimate diagnosis of this uncommon and aggressive tumor. The lesion occurred in a 79 year old patient. Histologically, the tumor was composed of intradermal small cells with high mitotic index and frequent apoptosis. The immunohistochemical study showed positivity for wide spectrum and low molecular weight cytokeratins, epithelial membrane antigen, neurofilaments, neuron specific enolase and chromogranin A. Electron microscopy revealed intermediate filaments in a typical globular paranuclear arrangement. The coexpression of cytokeratins (including cytokeratin 20) and neurofilaments, both in typical globular paranuclear arrangement, made possible the diagnosis of MCC, differentiating it from other malignant small cell tumors such as neuroendocrine metastatic carcinoma.

Breast cancer screening of the high risk population with clinical examination and thermography. A combination of telethermography and plate thermography.

On a total of 2523 patients, including the High Risk group, a breast screening was made by clinical exploration and Thermography used as a selective method for the cases due for a Mammography. Although the electronic thermographic examination was done according to the criteria established by the School of Marseilles, we excluded from these criteria the vascular asymmetry test. Such a controversial parameter was substituted by the data given by the angiographic analysis of contact thermography. The results obtained were compared to those referenced in a previous study. The analysis of the compiled data indicated that even if we weren 't the sensitivity of the technique, we did get improvements diagnosing less false positive results with the new procedure (91.43%) than with the previous study (87.65%) (p less than 0.001). The predictive Value (+) was 0.4133 in the present group against the previous 0.10, which meant that 41.33% of the cancers diagnosed were true cancers, against 10% in the previous group. Summing up the analysis showed a higher rate of positive global detections (p less than 0,0005) with our present analytic criteria for Thermography.

[Infections due to herpes-varicella viruses in Hodgkin's disease (author's transl)]. / Infecciones por herpes virus-varicela en la enfermedad de Hodgkin.

The infections due to herpes-varicella viruses occurring in 191 patients with Hodgkin's disease form the basis of this report. There were overall 41 episodes (26.7%) in 40 patients, distributed as follows: varicella in three cases, atypical herpes-varicella in two cases, and herpes zoster in 36 cases, the latter showing systemic spread in seven instances, one to the central nervous system (myelitis) and six to the skin. The mortality was 2.5% of all infections, and 33% of the varicella cases. Morbidity was apparent as postherpetic neuralgia in seven patients (19.4%), postherpetic paraplegia in one case (2.5%), and severe thrombocytopenia in another case (2.5%). The statistical study of the factors contributing to the development of reactivation episodes demonstrated that neither age, sex, or previous splenectomy were influential. The results obtained in relation to the stage and histologic type of Hodgkin's disease can not be fully evaluated because of the artifact introduced by other variables such as type of therapy and observation time. There was a clear relationship with the aggressiveness of therapy, because 81.7% of the viral episodes occurred in patients submitted to total radiotherapy with or without chemotherapy, or with partial radiotherapy plus chemotherapy. In the patients with systemic spread there was a clear relationship with prior splenectomy (p less than 0.005). The clinical features of these patients are commented upon.

[Primary Sjogren's syndrome in men: evolution to acute lymphoblastic leukemia]. / Síndrome de Sjögren primario en varón: evolución a leucemia linfoblástica aguda.

A clinical case of a 55-year-old man with syndrome of primary Sjögren, seronegative, normoglobulinemic and normocomplementary with 7 years of evolution, which developed acute lymphoblastic leukemia, having suffered recurrent lympho-adenopathy and paresthetic meralgia as previous clinical signs, is presented. Clinical, serological and immunologic characteristics of this syndrome are reviewed, highlighting its rare appearance among men. While appearance of malignant lymphoproliferation disorders is well known, evolution to acute or chronic myeloid leukemia is very rare and so far, an acute lymphoblastic leukemia as the one presented in this study has never been described.

[A prognostic multifactorial study of sepsis in an internal medicine service]. / Estudio multifactorial pronóstico de la sepsis en un Servicio de Medicina Interna.

All cases of sepsis attended at the Service of Internal Medicine from 1985 to 1989, both inclusive, were retrospectively analyzed by the statistical study of several clinical, epidemiological, bacteriological and laboratory factors. The parameters acting as prognosis factors were analyzed using Cox's method of logistic regression, in order to obtain more reliable information on the multifactorial spectrum determining "death due to sepsis". The unifactorial analysis (UFA) suggested that the following factors were associated to an statistically significant increase in the mortality due to sepsis: age above 70; male sex; presence of shock upon hospitalization; nosocomial etiology; high levels of urea, creatinine and LDH; proteinuria; family of the causal germ (gram-positive coccus) and underlying pathology. The relative risks (RR) or "odd ratios" of creatinine and LDH were, respectively, 2.8 and 2.9 in the UFA; 3 and 3.3 in the multivariant analysis (MVA). In the older patients developing sepsis within their communities, RR were respectively 2.7 and 1.98 in the UFA and 1.1 and 2.7 in the MFA. The results of the univariant and multivariant analysis showing the relative risk (odd ratio) associated to each of these variables with statistical significance, demonstrate that the mortality rate due to sepsis increases with age, nosocomial etiology and elevation of creatinine and lactodehydrogenase (LDH) levels. We stress the great importance of the LDH as a prognostic factor of sepsis.

[The solitary pulmonary nodule. An analysis of the experience over 6 years in a medical service]. / Nódulo pulmonar solitario. Análisis de la experiencia durante 6 años en un servicio médico.

We conducted a retrospective analysis of the epidemiological, etiological, clinical and radiological characteristics, and mainly of those related to the diagnosis and treatment, of all the patients with radiological criteria of solitary pulmonary nodule (SPN) studied in our environment during a period of six years (1984-1989). The incidence of SPN was 3.1% (123/3953). Among the 117 cases of filiated etiology, 83 (71%) were malignant and 34 (29%), benign thoracotomy was used as diagnostic method in 11 (32%) benign cases and in 8 (9.6%) malignant cases. Due to several causes, only 31 out of the 75 (41.3%) malignant nodules diagnosed prior to the thoracotomy underwent resection surgery. According to the selection criteria applied in our medium, a high number of the SPN were malignant; in most of these cases, the diagnosis was established without the need of thoracotomy and in less that half of them, curative surgery was attempted.

Frasier syndrome: A case report / El síndrome de Frasier, caso clínico

Introduction: Frasier syndrome is a genetic disorder produced by a mutation in intron 9 of the WT1 gene, responsible for renal and genital dysfunctions. Clinical findings: It is characterized by discrepancy between the individual karyotype and the individual phenotype and corticosteroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis. Patients usually have a female phenotype with a 46 XY karyotype, which increases the risk of gonadoblastoma in 50% of cases. Kidney disease requires kidney transplantation in adulthood. Cardiovascular and bone-derived comorbidities such as hyperlipidaemia and osteopenia/osteoporosis, respectively, are also common. Main diagnoses: Mutations of the WT1 gene can lead to different clinical entities, most notably Denysh-Drash syndrome, Frasier syndrome, or isolated focal segmental glomerulosclerosis. We present a clinical case of a woman who debuted in childhood with difficult-to-control nephrotic syndrome, the lack of pubertal development, primary amenorrhoea and the absence of ovaries on imaging tests in adolescence, alerted to an underlying genetic problem that, after cytogenetic studies, allowed a diagnosis of Frasier syndrome. Therapeutic interventions: It is recommended to remove the gonads due to increased risk of developing gonadoblastoma. Treatment of associated dyslipidaemia and osteopenia is also necessary. Conclusion: Frasier syndrome is an unusual cause of infertility due to gonadal dysgenesis and is associated with kidney problems.(AU)

Introducción: El síndrome de Frasier es un trastorno genético producido por una mutación en el intrón 9 del gen WT1, responsable de disfunciones a nivel renal y genital. Principales síntomas: Se caracteriza por disgenesia gonadal con discrepancia entre cariotipo-fenotipo y síndrome nefrótico resistente a corticoides debido a glomeruloesclerosis focal y segmentaria. Las pacientes presentan habitualmente fenotipo femenino con cariotipo 46 XY, lo que aumenta el riesgo de gonadoblastoma en un 50% de los casos. La enfermedad renal obliga a trasplante renal en la edad adulta. Son habituales también las comorbilidades derivadas a nivel cardiovascular y óseo como hiperlipidemia y osteopenia/osteoporosis, respectivamente. Diagnósticos principales: Las mutaciones del gen WT1 pueden conducir en distintas entidades clínicas entre las que destaca el síndrome de Denys-Drash, el síndrome de Frasier o la glomeruloesclerosis focal y segmentaria aislada. Se presenta un caso clínico de una mujer que debutó en la infancia con síndrome nefrótico de difícil control y que, durante la adolescencia, ante la falta de desarrollo puberal, la amenorrea primaria y la ausencia de ovarios en las pruebas de imagen alertaron de un problema genético subyacente que, tras estudios citogenéticos, permitió el diagnóstico de síndrome de Frasier. Intervenciones terapéuticas: Se recomienda la exéresis de las gónadas debido al riesgo incrementado de gonadoblastoma. El tratamiento de la dislipemia y la osteopenia asociadas también es necesario. Conclusión: El síndrome de Frasier es una causa inusual de infertilidad debido a una disgenesia gonadal y se asocia con problemas a nivel renal.(AU)

Tumor trofoblástico del lecho placentario: reporte de un caso / Placental site trophoblastic tumour: A case report

El tumor trofoblástico del lecho placentario es una afección muy infrecuente que forma parte del grupo de las neoplasias trofoblásticas gestacionales. Se origina generalmente tras un aborto o gestación normal a partir de la proliferación patológica del trofoblasto intermedio extravelloso, que puede ocurrir en meses o años tras la gestación. Su presentación clínica habitual es el sangrado uterino anómalo, la amenorrea y unos niveles de β-hCG bajos, en contra de lo que ocurre con el coriocarcinoma o la mola invasiva. El tratamiento de elección es la histerectomía junto con linfadenectomía pélvica. Es particularmente resistente a la quimioterapia, y un 30% de las pacientes presentan metástasis al diagnóstico. Se presenta un caso clínico de un tumor trofoblástico del lecho placentario diagnosticado en nuestro centro en julio de 2018, en una paciente con infertilidad que tras 3 abortos espontáneos comenzó con unos niveles de β-hCG bajos, pero en meseta y con tendencia al alza. Se aborda el enfoque diagnóstico y terapéutico que recibió, así como la evolución hasta la actualidad.(AU)

Placental site trophoblastic tumour (PSTT) is a very rare pathology that is part of the group of gestational trophoblastic neoplasia (GTN). It generally develops after a nonmolar abortion or term pregnancy from the pathological proliferation of extravillous intermediate trophoblast that can occur months or years after gestation. Its usual clinical presentation is abnormal uterine bleeding, amenorrhoea, and low levels of β-hCG, in contrast to choriocarcinoma or invasive mole. The treatment of choice is hysterectomy along with pelvic lymphadenectomy. It is particularly resistant to chemotherapy and 30% of patients present metastatic disease on diagnosis. A clinical case of PSTT diagnosed at our centre in July 2018 in a patient with infertility who, after 3 spontaneous abortions, debuted with low levels of β-hCG but on a plateau and with an upward trend. The diagnostic and therapeutic approach she received is addressed, as well as the evolution to date.(AU)

Leiomiosarcomas de grandes vasos en las extremidades inferiores / Leiomyosarcomas affecting main vessels in the lower extremities

Objetivos: Evaluar los resultados de la resección en bloque y la reconstrucción vascular de leiomiosarcomas con afectación de vasos principales en las extremidades inferiores. Material y métodos: Desde enero de 1983 a diciembre 2016 se diagnosticaron 42 pacientes con leiomiosarcomas, de los que 6 afectaban a vasos principales de las extremidades inferiores (denominados vasculares). Se registraron retrospectivamente datos epidemiológicos, estudios de imagen, cirugía realizada, tratamientos adyuvantes, complicaciones, así como las recidivas y mortalidad. Resultados: Todos los pacientes fueron afectos de leiomiosarcomas de alto grado(II-III FNCLCC), con un diámetro mayor tumoral de 9,1cm de media (6-15) y un seguimiento medio de 23 meses (7-36). La edad media fue de 64 años (29-84). El primer síntoma fue una tumoración palpable en 4 de ellos. Los otros 2 casos comenzaron con fenómenos tromboembólicos. En 5 casos el origen fueron los vasos femorales, mientras que un caso fue a nivel poplíteo. A pesar de que todos los casos preservaban la extremidad, 3 (50%) presentaron diseminación pulmonar, 2 (33%) hepática y un caso presentó recidiva local. Dos casos fueron exitus al finalizar el estudio y hubo un caso de pérdida de seguimiento. Discusión y conclusiones: Los leiomiosarcomas vasculares son tumores altamente agresivos con baja tasa de supervivencia a los 5 años. En nuestro estudio, el 50% de los pacientes se mantienen en remisión completa con un seguimiento medio de 23 meses. Es frecuente que su inicio asocie la presencia de una masa tumoral con fenómenos trombóticos (33% de nuestros casos). La cirugía de resección tumoral habitualmente compromete las estructuras vasculares principales, hecho que implica una resección y técnicas reconstructivas vasculares para el salvamento de la extremidad

Objective: To evaluate the results of bloc resection and vascular reconstruction of leiomyosarcomas with involvement of main vessels in the lower extremities. Material and methods: From January 1983 to December 2016, 42 patients with leiomyosarcomas were diagnosed. Six of these leiomyosarcomas affected main vessels of the lower extremities (called vascular). Epidemiological data, imaging studies, surgery performed, adjuvant treatments, complications, as well as recurrences and mortality were retrospectively recorded. Results: All the patients were affected by high-grade leiomyosarcomas (II-III FNCLCC classification), with a larger tumour average diameter of 9.1cm(6-15) and a mean follow-up of 23 months (7-36). The average age was 64 years (29-84). The first symptom was a palpable tumour in 4 of them. The other 2 cases debuted with thromboembolic phenomena. In 5 cases the origin was the femoral vessels, while one case was at the popliteal level. Although all cases preserved the limb, in 3 cases (50%) they presented pulmonary dissemination,2 cases (33%) hepatic dissemination and one case had local recurrence. Two cases died at the end of the study and there was one case of loss to follow-up. Discussion and conclusions: Vascular leiomyosarcomas are highly aggressive tumours with a low survival rate at 5 years. In our study, 50% of the patients remain in complete remission with a mean follow-up of 23 months. Their onset frequently associates the presence of tumour mass with thrombotic phenomena (33% of our cases). Tumour resection surgery usually compromises the main vascular structures, which implies resection and vascular reconstructive techniques to salvage the limb