Effect of emergent nephrostomy on long-term total and split renal function in patients with upper urinary tract obstruction due to pelvic malignant tumors.

PURPOSE: This study aimed to investigate the impact of nephrostomies on the outcome of total renal function (TRF) and split renal function (SRF) in patients with malignant pelvic tumors associated with upper urinary tract obstruction (UUTO). METHODS: Patients with pelvic tumors suffering severe unilateral hydronephrosis treated at our hospital from 2000 to 2022 were included. Data for nephrostomy placement, short- and long-term renal function, and radiological and nuclear imaging studies were collected. The TRF and SRF of patients who underwent nephrostomy were compared to those who did not. RESULTS: Seven patients were included (rhabdomyosarcoma: 5, ovarian germ cell tumor: 1, malignant rhabdoid tumor: 1). Nephrostomies were placed in four, which were successfully managed without severe infections. Estimated glomerular filtration rate (eGFR) was significantly improved at the end of treatment in patients with nephrostomy. In contrast, eGFR in patients who did not undergo nephrostomy was not improved. Nuclear imaging studies (renograms or renal scintigrams) revealed impaired SRF of the affected kidney compared to the contralateral kidney, even in patients whose eGFR was within normal levels. Notably, SRF showed a trend to improve over time in one patient treated with nephrostomy. CONCLUSION: Nephrostomy for UUTO caused by pelvic tumors may improve renal outcome.

Robotic-assisted laparoscopic resection of a pelvic solitary fibrous tumour causing distal ureteric compression.

This case report describes a male in his late 40s with a 4 cm pelvic mass compressing the left distal ureter, resulting in left hydroureteronephrosis. Biopsy of the mass was suggestive of a solitary fibrous tumour. The patient underwent a robotic-assisted laparoscopic excision of the left pelvic mass. Intraoperatively, the mass was found to be densely adhered to the ureter, necessitating a left distal ureterectomy and ureteric reimplantation. Subsequent histopathological analysis revealed the mass was a solitary fibrous tumour with no evidence of malignancy.

Sympathetic nerve blocks for persistent pain in adults with inoperable abdominopelvic cancer.

BACKGROUND: Persistent visceral pain is an unpleasant sensation coming from one or more organs within the body. Visceral pain is a common symptom in those with advanced cancer. Interventional procedures, such as neurolytic sympathetic nerve blocks, have been suggested as additional treatments that may play a part in optimising pain management for individuals with this condition. OBJECTIVES: To evaluate the benefits and harms of neurolytic sympathetic nerve blocks for persistent visceral pain in adults with inoperable abdominopelvic cancer compared to standard care or placebo and comparing single blocks to combination blocks. SEARCH METHODS: We searched the following databases without language restrictions on 19 October 2022 and ran a top-up search on 31 October 2023: CENTRAL; MEDLINE via Ovid; Embase via Ovid; LILACS. We searched trial registers without language restrictions on 2 November 2022: ClinicalTrials.gov; WHO International Clinical Trials Registry Platform (ICTRP). We searched grey literature, checked reference lists of reviews and retrieved articles for additional studies, and performed citation searches on key articles. We also contacted experts in the field for unpublished and ongoing trials. Our trial protocol was preregistered in the Cochrane Database of Systematic Reviews on 21 October 2022. SELECTION CRITERIA: We searched for randomised controlled trials (RCTs) comparing any sympathetic nerve block targeting sites commonly used to treat abdominal pelvic pain from inoperable malignancies in adults to standard care or placebo. DATA COLLECTION AND ANALYSIS: We independently selected trials based on predefined inclusion criteria, resolving any differences via adjudication with a third review author. We used a random-effects model as some heterogeneity was expected between the studies due to differences in the interventions being assessed and malignancy types included in the study population. We chose three primary outcomes and four secondary outcomes of interest. We sought consumer input to refine our review outcomes and assessed extracted data using Cochrane's risk of bias 2 tool (RoB 2). We assessed the certainty of evidence using the GRADE system. MAIN RESULTS: We included 17 studies with 1025 participants in this review. Fifteen studies with a total of 951 participants contributed to the quantitative analysis. Single block versus standard care Primary outcomes No included studies reported our primary outcome, 'Proportion of participants reporting no worse than mild pain after treatment at 14 days'. The evidence is very uncertain about the effect of sympathetic nerve blocks on reducing pain to no worse than mild pain at 14 days when compared to standard care due to insufficient data (very low-certainty evidence). Sympathetic nerve blocks may provide small to 'little to no' improvement in quality of life (QOL) scores at 14 days after treatment when compared to standard care, but the evidence is very uncertain (standardised mean difference (SMD) -0.73, 95% confidence interval (CI) -1.70 to 0.25; I² = 87%; 4 studies, 150 participants; very low-certainty evidence). The evidence is very uncertain about the risk of serious adverse events as defined in our review as only one study contributed data to this outcome. Sympathetic nerve blocks may have an 'increased risk' to 'no additional risk' of harm compared with standard care (very low-certainty evidence). Secondary outcomes Sympathetic nerve blocks showed a small to 'little to no' effect on participant-reported pain scores at 14 days using a 0 to 10 visual analogue scale (VAS) for pain compared with standard care, but the evidence is very uncertain (mean difference (MD) -0.44, 95% CI -0.98 to 0.11; I² = 56%; 5 studies, 214 participants; very low-certainty evidence). There may be a 'moderate to large' to 'little to no' reduction in daily consumption of opioids postprocedure at 14 days with sympathetic nerve blocks compared with standard care, but the evidence is very uncertain (change in daily consumption of opioids at 14 days as oral milligrams morphine equivalent (MME): MD -41.63 mg, 95% CI -78.54 mg to -4.72 mg; I² = 90%; 4 studies, 130 participants; very low-certainty evidence). The evidence is very uncertain about the effect of sympathetic nerve blocks on participant satisfaction with procedure at 0 to 7 days and time to need for retreatment or treatment effect failure (or both) due to insufficient data. Combination block versus single block Primary outcomes There is no evidence about the effect of combination sympathetic nerve blocks compared with single sympathetic nerve blocks on the proportion of participants reporting no worse than mild pain after treatment at 14 days because no studies reported this outcome. There may be a small to 'little to no' effect on QOL score at 14 days after treatment, but the evidence is very uncertain (very low-certainty evidence). The evidence is very uncertain about the risk of serious adverse events with combination sympathetic nerve blocks compared with single sympathetic nerve blocks due to limited reporting in the included studies (very low-certainty evidence). Secondary outcomes The evidence is very uncertain about the effect of combination sympathetic nerve blocks compared with single sympathetic nerve blocks on participant-reported pain score and change in daily consumption of opioids postprocedure, at 14 days. There may be a small to 'little to no' effect, but the evidence is very uncertain (very low-certainty evidence). There is no evidence about the effect on participant satisfaction with procedure at 0 to 7 days and time to need for retreatment or treatment effect failure (or both) due to these outcomes not being measured by the studies. Risk of bias The risk of bias was predominately high for most outcomes in most studies due to significant concerns regarding adequate blinding. Very few studies were deemed as low risk across all domains for any outcome. AUTHORS' CONCLUSIONS: There is limited evidence to support or refute the use of sympathetic nerve blocks for persistent abdominopelvic pain due to inoperable malignancy. We are very uncertain about the effect of combination sympathetic nerve blocks compared with single sympathetic nerve blocks. The certainty of the evidence is very low and these findings should be interpreted with caution.

The effect of psychosocial interventions for sexual health in patients with pelvic cancer: a systematic review and meta-analysis.

AIM: The aim of this systematic review and meta-analysis was to explore and evaluate the effect of psychosocial interventions in improving sexual health outcomes among post-treatment patients with pelvic cancer. METHODS: Inclusion and exclusion criteria were pelvic cancer survivors; psychosocial interventions; studies with a control group and measures of sexual health. Five databases were searched for literature along with an inspection of the included studies' reference lists to extend the search. Risk of bias was assessed with the RoB2 tool. Standardised mean difference (SMD) with a random effects model was used to determine the effect size of psychosocial interventions for sexual health in patients with pelvic cancers. RESULTS: Thirteen studies were included, with a total number of 1,541 participants. There was a large heterogeneity regarding the type of psychosocial intervention used with the source found in a leave one out analysis. Six studies showed statistically significant improvements in sexual health, while three showed positive but non-significant effects. The summary effect size estimate was small SMD = 0.24 (95% confidence interval [CI]: 0.05 to 0.42, p = 0.01). DISCUSSION: There is limited research on psychosocial interventions for sexual health in pelvic cancer patients. There are also limitations in the different pelvic cancer diagnoses examined. Commonly, the included articles examined physical function rather than the whole sexual health spectrum. The small effect sizes may in part be due to evaluation of psychosocial interventions by measuring physical dysfunction. Future research should broaden sexual health assessment tools and expand investigations to more cancer types.

Association between types of abdominopelvic cancer in patients with situs inversus total: Systematic review.

BACKGROUND: Situs inversus is a rare congenital anatomical variant that involves a group of anomalies regarding the arrangement of intrathoracic and intraabdominal organs. Being able to find in the abdominal region the liver, gallbladder, inferior vena cava, and head of the pancreas and ascending colon on the left side of the abdomen, while on the right side there is the spleen, the stomach, the body of the pancreas, the ligament of Treitz, descending colon among others. In this same way, the thoracic organs, lungs and heart, are changed in their position in a mirror translocation. METHODS: We systematically searched MEDLINE, Web of Science, Google Scholar, CINAHL, Scopus, and LILACS; the search strategy included a combination of the following terms: "Situs inversus," "Situs inversus totalis," "Cancer," "Neoplasm," "Abdominopelvic regions," and "clinical anatomy." RESULTS: Within the 41 included studies, 46 patients with situs inversus who had cancer, in addition to being found in this organ and in these regions, we also found as a result that the majority of the studies in the research were in stage II; finally, no one study could assert the direct relationship between the situs inversus totalis and the cancer. CONCLUSION: If our hallmarks could make us think that more exhaustive follow-up of the stomach and other organs should be carried out in these patients, there could also be other predisposing factors for cancer, which is why more studies are suggested to give future diagnostic and treatment guidelines treatment.

Effectiveness of the superior hypogastric plexus neurolytic block in the management of pelvic abdominal pain in adult patients diagnosed with cancer: A systematic literature review / Efectividad del bloqueo neurolítico del plexo hipogástrico superior en el manejo del dolor abdominopélvico en pacientes adultos con diagnóstico de cáncer. Revisión sistemática de la literatura

INTRODUCTION: Severe oncological pain occurs in up to 60% of pelvic abdominal cáncer patients, being refractory to medical management in up to 30% of cases. In 1990, the superior hypogastric plexus neurolytic block (SHPB) was described for the control of pain in these patients. This study aimed to evaluate the effectiveness of this technique for the control of oncological pain. METHODOLY: Studies that evaluated the effectiveness of the SHPB using the classic or transdiscal approach in adult patients with oncological abdominal-pelvic pain were systematically reviewed. A search was conducted in PubMed, EMBASE and Scopus from January 1, 1990, to August 31, 2019, without a language restriction. The visual analog scale (VAS), morphine milligram equivalents (MME) per day, quality of life and presence of complications were recorded. The quality of the studies was evaluated using the Jadad and Ottawa-Newcastle scales. RESULTS: Eight studies met the inclusión criteria: 6 were descriptivo longitudinal studies, and 2 were controlled clinical trials, comprising 316 patients (75% female and 25% male; average age 53.2 years); the most frequent diagnoses were gynecological (65%) cancer. An average VAS reduction of 55%-60.8% was obtained as well as a MME reduction of 40%-60%. Three studies evaluated the quality of life using the (QLQ-C30), (PSS) and Zubrod scale all with positive results. Complications were reported in 18% of cases, pain related to the puncture was the most frequent. CONCLUSIONS: The SHPB may be an effective for the control of severe oncological abdominal-pelvic pain, decreasing the VAS and MME and improving the quality of life of patients.

INTRODUCCIÓN: El dolor oncológico severo se presenta hasta en el 60% de los pacientes con cáncer abdominopélvico, siendo refractario al manejo médico hasta en el 30% de los casos. En 1990, se describió el bloqueo del plexo hipogástrico superior (BPHS) para el control de dolor en estos pacientes. Nuestro objetivo en este estudio fue evaluar la efectividad de esta técnica. METODOLOGÍA: Se realizó una revisión sistemática de estudios que evaluaron la efectividad del BPHS técnicas guiadas por fluoroscopio en pacientes adultos con dolor oncológico abdominopélvico. Se realizó una búsqueda en Pubmed, EMBASE y Scopus desde el 1de enero de 1990 hasta el 31 de agosto de 2019, sin restricción de idioma. Se evaluó la escala visual análoga, el consumo de opioides: dosis equivalente de morfina día, calidad de vida, presencia de complicaciones y se evaluó la calidad de los estudios mediante escalas Jadad y Ottawa- Newcastle. RESULTADOS: Ocho estudios cumplieron los criterios de inclusión, 6 fueron longitudinales descriptivos y 2 ensayos clínicos controlados, con un total de 316 pacientes, 75% femenino y 25% masculino; edad promedio 53,2 años; diagnóstico más frecuente: Cáncer ginecológico (65%). Se logró una reducción de la escala visual análoga (EVA) de 55%-60,8% y reducción de la dosis equivalente de miligramos de morfina oral día (DEMO) del 40%-60%. Tres estudios evaluaron la calidad de vida con las escalas QLQ-C30, PSS y Zubrod, mostrando mejoría en todas. Se reportaron complicaciones en 18% de los casos, siendo el dolor en el sitio de punción la más frecuente.CONCLUSIONES: El BPHS puede ser efectivo en el control de dolor oncológico de origen abdominopélvico, disminuyendo escala visual análoga (EVA), dosis equivalente de miligramos de morfina oral día (DEMO) y mejorando la calidad de vida. Sin embargo, se requieren de estudios adicionales para dar una recomendación con alta calidad de evidencia.

Ureteropelvic junction obstruction caused by metastatic cholangiocarcinoma

ABSTRACT We describe the rare case of a 61-year-old female with right ureteropelvic junction (UPJ) obstruction caused by metastatic cholangiocarcinoma. Her past medical history was notable for cholangiocarcinoma treated with neoadjuvant chemoradiation and two orthotopic liver transplants six years earlier. Urology was consulted when she presented with flank pain and urinary tract infection. Diagnostic workup demonstrated right UPJ obstruction. She was managed acutely with percutaneous nephrostomy. She subsequently underwent robotic pyeloplasty and intrinsic obstruction of the UPJ was discovered. Histological examination revealed adenocarcinoma, consistent with systemic recurrence of the patient's known cholangiocarcinoma.

Angiomixoma pélvico asociado a endometriosis infiltrativa profunda: reporte de un caso / Pelvic angiomyxoma associated to deep infiltrative endometriosis: case report

El angiomixoma de la pelvis es un tumor mesenquimal inusual; que dado su alto potencial de recidiva es relevante que la exéresis quirúrgica sea lo más radical posible. Por su parte, la endometriosis infiltrativa profunda, es otra entidad poco frecuente que requiere de tratamientos complejos. La co-existencia de ambas patologías representa una situación absolutamente infrecuente; de los que hay muy pocos casos reportado en la literatura occidental. Se presenta el caso de una mujer de 41 años de edad que desarrolló ambas entidades nosológicas de forma concomitante y que fue tratada quirúrgicamente con buenos resultados.

Angiomyxoma of the pelvis is an unusual mesenchymal tumor; that given its high potential for recurrence, it is relevant that the surgical resection be as radical as possible. For its part, to deep infiltrative endometriosis is another rare entity that requires complex treatments. The co-existence of both pathologies represents an absolutely infrequent situation; of which there are very few cases reported in western literature. We present the case of a 41-year-old woman who developed both clinical entities concomitantly and who was treated surgically with good results.

Pelvic chondro-sarcoma and erectile dysfunction: a challenging surgical case / Chondro-sarcoma pélvico y la disfunción eréctil: un desafiante caso quirúrgico

A 46 year-old male presented suffering from refractory erectile dysfunction. The patient had a past history of pelvic chondro-sarcoma. This case was a challenge for us; the surgery performed for excision of the sarcoma has resulted in the elimination of both pubic ramia together with excision of part of both corpora and left hemiscrotum. The procedure included a sling application on one side with a mesh sock wrapped around the rear part of the extendor cylinder. Another sling was applied on the contra lateral side without a mesh sock (AU)

Varón de 46 años que presenta el sufrimiento por disfunción eréctil refractaria. El paciente tenía antecedentes de condro sarcoma pélvico. Este caso fue un reto para nosotros; la cirugía realizada para la extirpación del sarcoma ha dado como resultado la eliminación de ambas ramas púbicas junto con la extirpación de parte de ambos corpus y hemisrcotum izquierdo. El procedimiento incluyó una aplicación de eslinga en uno de los lados con una funda de malla envolviendo la parte trasera del cilindro extensor. Se aplicó otra eslinga en el lado contra lateral sin una funda de malla (AU)

Tratamiento de la hemorragia presacra tras cirugía colorrectal mediante el uso del balón de Bakri / Treatment of presacral bleeding after colorectal surgery with Bakri balloon

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Metástasis pélvicas de cáncer de mama: revisión de cuatro casos / No disponible

La pelvis es una localización infrecuente de metástasis de cáncer de mama siendo los ovarios el sitio de afectación más común. El diagnóstico y tratamiento de las masas pélvicas de un cáncer de mama es difícil y a menudo una manifestación de la progresión metastásica de la enfermedad. En pacientes con antecedentes de cáncer de mama, la laparoscopia exploratoria parece ser la vía de elección para el diagnóstico histológico y valoración de la resecabilidad de las lesiones. Presentamos cuatro casos de metástasis de cáncer de mama en ovarios y útero y se discute el comportamiento, tratamiento y evolución de las pacientes con metástasis pélvicas(AU)

The pelvis is an uncommon metastatic site for breast cancer and the ovarian origin is the common site. The diagnosis and treatment of pelvic masses from a metastatic breast cancer are difficult and often a manifestation of metastatic progression in a patient with a known history of breast cancer. In patients with history of breast cancer, laparoscopy seems to be the preferred technique to establish the histological diagnosis and to assess the resectability of the lesions. We present four cases of metastatic breast cancer in the pelvis to the uterus and ovarian and discuss the treatment and prognosis of the patients with pelvic metastasis(AU)

Late gastrointestinal complications after pelvic radiotherapy: radiation enteritis

INTRODUCTION: Radiation enteritis is a complication of radiation therapy for pelvic tumours. It appears after a variable period of time and is often progressive. MATERIAL AND METHODS: We analyse our experience of 77 cases (52 females and 25 males) diagnosed with radiation enteritis or proctitis between 1986 and 2006. RESULTS: The most frequent location of radiation injury is ileum (55 patients, 71%), followed by rectum (22 patients, 28%). Twenty-eight patients (36%) were medically managed and 49 (64%) required surgical treatment. In 41 (53%) of the patients the affected region was resected, in 5 (7%) a by-pass was performed and in 3 (4%) a terminal colostomy. Surgical mortality was 4% (3 cases) and the complication rate 9% (7 cases). Twelve patients (16%) presented recurrence of radiation-related illness. Excluding those cases deceased because of tumoral progression, 5-year survival rate was 90% and 10-year survival rate 83%. CONCLUSION: Radiation enteritis must be initially conservatively managed, but in those cases without response, surgery is indicated. Surgical treatment should not be delayed fearing postoperative complications, which are more susceptible to appear in deteriorated patients. If technically possible, the affected region should be resected, because complications may appear later at this damaged location (AU)

Manejo de los tumores retrorrectales del adulto / Management of the retrorectal tumors of the adult

Antecedentes: Los tumores que asientan en el espacio virtual entre el mesorrecto y el sacrocoxis comprenden un grupo heterogéneo y poco frecuente cuya incidencia se estima en 1/40.000 ingresos. Se clasifican en congénitos, neurogénicos, óseos y misceláneas. Por ser asintomáticos u ocasionar síntomas inespecíficos su diagnóstico suele ser tardío y muchas veces cuando han alcanzado un gran tamaño o comprometido las estructuras vecinas. El diagnóstico y tratamiento, que requiere un equipo multidisciplinario, han evolucionado en los últimos años por el aporte de la resonancia magnética nuclear (RMN), las nuevas terapias quimiorradiantes y un abordaje quirúrgico más agresivo. Objetivo: Comunicar nuestra experiencia y sugerir la estrategia de manejo de estos tumores basada en esta serie y la de los centros internacionales de referencia. Pacientes y Métodos: Se revisaron retrospectivamente las historias clínicas de los pacientes con tumores retrorrectales operados entre 1991 y 2006 en la División Cirugía del Hospital Juan A. Fernández. Se excluyeron procesos inflamatorios, tumores rectales localmente avanzados y metastásicos. Se registraron sexo, edad, síntomas/signos, tiempo de evolución, estudios preoperatorios, tamaño tumoral, compromiso sacro y/o de órganos vecinos, tratamiento quirúrgico, morbimortalidad inmediata, estadía postoperatoria, secuelas, histopatología, recurrencia y supervivencia. Además se evaluó la utilidad de la tomografía axial computada (Te) y la RMN para establecer la estirpe tumoral, la posible malignidad, la invasión de estructuras vecinas y la vía de abordaje. Resultados: Hubo 7 pacientes (5 mujeres), edad promedio 37,5 (23-54) años. Congénitos: 2 (cordoma 1, hamartoma quístico 1), neurogénicos: 2 (neurofibroma plexiforme 1, schwanoma maligno 1), óseos: 1 (tumor de células gigantes del sacro) y misceláneas: 2 (liposarcoma 1, fibroma extrapleural maligno 1)...

Background: Tumors occupying the virtual space between the mesorectum and sacro-coccyx are heterogeneous and infrequent, with an estimated incidence of 1/40.000 hospitalizations. They are classified as congenital, neurogenic, osseous, and miscellaneous. Because they are asymptomatic or cause non-specific symptoms diagnosis is usually delayed, and very often done when have reached a great dimension or involved adjacent structures. Diagnosis and treatment, that required a multidisciplinary team, has evolved in recent years due to the role of magnetic resonance imaging (MRI), new chemo-radiation therapies and a more aggressive surgical approach. Objective: Report on our experience, and suggest the management strategy for these tumors based on this series and that of international referral centers. Patients and Methods: Clinical records of patients with retrorectal tumors operated on, between 1991 and 2006 in the Division of Surgery of the Hospital Juan A. Fernández were retrospectively reviewed. Inflammatory processes, locally advanced rectal tumors and metastatic lesions were excluded. Registe red data included gender, age, symptom/signs, time of evolution, preoperative studies, size of tumors, involvement of sacrum and/or adjacent viscera, surgical treatment, 30-day morbidity and mortality, postoperative hospital stay, secuela, histopathology, recurrence and survival. Besides, the usefulness of computed tomography (CT) and MRI to establish the histologic tumor type, possible malignancy, invasion of adjacent structures, and operative approach was assessed. Results: Seven patients (5 women), mean age 37.5 (23-54) years, were treated. Congenital: 2 (chordoma 1, tailgut cyst 1), neurogenic: 2 (plexiform neurotibroma 1, malignant schwannoma 1), osseous: 1 (gigant cell tumor of the sacrum) and miscellaneous: 2 (liposarcoma 1, extrapleural malignant fibroma 1)...

Pecoma pélvico del ligamento ancho con metástasis ganglionares: presentación de un caso y revisión de la literatura / Pelvic PEcoma of the broad ligament with lymph node metastasis: case report and bibliographic review

Objetivos: Describimos y documentamos el primer caso de PEComa publicado en España según la base de datos Pub Med. Y revisamos la bibliografía existente sobre este tipo de tumores. Métodos/Resultados: Se Trata de una paciente de 39 años con un tumor pélvico de 9 cms. descubierto en una revisión ginecológica rutinaria. El TAC muestra múltiples adenopatías periaórticas que se extienden hasta vena renal izquierda El diagnóstico de PEComa se obtiene por punción con aguja gruesa. Se realiza exéresis del tumor junto a anexo izquierdo y linfadenectomía aortoiliaca. El resultado patológico confirma el diagnóstico tras la inmunohistoquímica con actina de músculo liso y HMB-45. No recibió tratamiento adyuvante. Tras un año de seguimiento la paciente no muestra en el TAC signos de recidiva. Se realiza una amplia búsqueda bibliográfica en Pub Med encontrando 73 referencias a este tipo de tumor cuyas conclusiones se exponen en este articulo. Conclusiones: El tumor de celulas epitelioides perivasculares, PEComa, es una neoplasia mesenquimal de muy baja incidencia y con malignidad incierta. Tumores muy raros: el angiomiolipoma epitelioide, la linfangioleiomiomatosis, el linfangiomioma, el tumor miomelanocitico del ligamento falciforme, el tumor pulmonar de celulas claras «de azucar» y su variante extrapulmonar; son antiguas descripciones de lo que ahora es una única entidad tumoral denominada PEComa. Aun no de ha descrito la célula que en tejidos normales da origen a este tumor. Afectando principalmente a mujeres en edad media, pueden encontrarse en cualquier lugar anatómico. Su tratamiento es quirúrgico aunque se desconoce el papel de la linfadenectomía y del tratamiento adyuvantes (AU)

Objective: We describe and document the first case of PEcoma published in Spain following the PubMed database. We review the bibliography about these tumors. Methods/Results: 39-year-old female patient with a 9 cm pelvic tumor discovered in a routine gynecologic review. CT scan showed multiple periaortic adenopathies extending up to the left renal vein. The diagnosis of PEcoma was obtained by needle biopsy. Tumor excision including left annex and aortoiliac lymphadenectomy were performed. Pathology confirmed the diagnosis after immunohistochemical study with smooth muscle actin and HMB-45. No adjuvant treatment was given. After one year of follow-up the patient does not show signs of recurrence of the CT scan. A PubMed search was performed finding 73 references about this kind of tumor the conclusions of which are exposed in this article. Conclusions: Perivascular epithelioid cell tumor, PEcoma, is a very low incidence mesenchymal neoplasia with uncertain malignancy. Very rare tumors such as epithelioid angiomyolipoma, lymphangioleiomyomatosis, lymphangiomyoma, myelomelanocytic tumor of the falciform ligament, «sugar» clear cell lung tumor and its extrapulmonary variant are all descriptions of what is now a unique tumoral entity named PEcoma. It has not been described yet the normal tissue cell giving origin to this tumor. It affects mainly middle age women, and may appear in any anatomical site. Treatment is surgical, although the role of lymphadenectomy and adjuvant treatment is unknown (AU)

Perihepatitis en adolescente sexualmente activa / Peri-hepatitis in a sexually active adolescent

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Angiomixoma agresivo de pelvis con afectación cardiaca / Aggressive angiomyxoma of the pelvis with cardiac involvement

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Obstrução urinária aguda por rabdomiossarcoma pélvico / Acute urinary obstruction due to pelvic rhabdomyosarcoma

Os autores relatam um caso raro de obstrução urinária aguda por rabdomiossarcoma pélvico envolvendo a próstata em uma criança de quatro anos e seis meses de idade. São comentados as características desses tumores, as formas de apresentação clínica, os aspectos diagnósticos por imagem — ultra-som, cistografia e tomografia computadorizada —, o tratamento com poliquimioterapia e o prognóstico.

The authors report a rare case of acute urinary obstruction caused by pelvic rhabdomyosarcoma in a 4-year-old children. The diagnosis aspects for imaging e some therapeutic considerations are related.

Lymphangiomyomatosis Arising in the Pelvic Cavity: A Case Report

Lymphangioleiomyomatosis (LAM) is a rare disease usually occurring in young women of child-bearing age. It is characterized by a distinctive proliferation of lymphatic smooth muscle cells, especially occurring in the pulmonary parenchyme. The majority of primary LAM occurs in the lung, but there are a few reports of extrapulmonary cases. We report a case of a 21-yr-old female who first complained of low abdominal pain and was referred from a local clinic with the impression of an ovarian cyst. Gynecologic ultrasonography revealed a large posterior pelvic mass with an irregular echogenicity measuring 9.7x4.2 cm in size. Pelviscopy showed a large, thin walled, partly cystic, pelvic mass. The mass was partly removed. Microscopically, the mass was characterized by a haphazard proliferation of smooth muscle cells arranged in fascicular, trabecular, and papillary patterns around a ramifying network of endothelium-lined spaces. The cells were plump or epithelioid with abundant eosinophilic cytoplasm and showed a positive reaction for both alpha-smooth muscle actin and HMB-45 antigen. Surgical and pathological findings were consistent with pelvic retroperitoneal LAM. Despite the numerous treatment attempts, the patient suffered from intractable chylous ascites and developed pulmonary LAM and died due to severe respiratory distress.

Tumores anexiales en pacientes embarazadas / Annexal tumors in pregnant women

Se trata de un estudio retrospectivo donde se analizaron 104 casos de pacientes embarazadas con tumoraciones anexiales en el Saint Barnabas Medical Center (Livingston, New Jersey, USA), entre el 1 de enero de 1982 y el 31 de diciembre de 1998. Esta serie fue comparada con las que ya han sido publicadas, analizando además las características histológicas y el manejo de dichas pacientes. (AU)