Pelvic Ewing Sarcoma: The Great Mimicker / Sarcoma de Ewing pélvico: o grande imitador

Abstract Ewing sarcoma is the most common malignant bone tumor of the pelvis in children and young adults. Even with aggressive treatment, its survival rate is amongst the poorest. Classical presentation may not be the rule. It may simulate clinically, imagiologically and histopathologically other nonmalignant entities. Therefore, its suspicion should not be overlooked. We report two cases of pelvic Ewing sarcoma: the first mimicking eosinophilic granuloma, and the second mimicking osteomyelitis. In the latter, we also report an atypical finding of its natural history: an initial response to antibiotic and anti-inflammatory treatment. In both cases, we highlight the possibility of an inconclusive percutaneous bone biopsy and the importance of immunochemistry and cytogenetics for the definitive diagnosis.

Resumo O sarcoma de Ewing é o tumor ósseo maligno da pelve mais comum em crianças e adultos jovens. Mesmo com tratamento agressivo, sua taxa de sobrevivência está entre as piores. A apresentação clássica pode não ser a regra. Ele pode simular clinicamente, imaginologicamente e histopatologicamente outras entidades não malignas. Portanto, sua suspeita não deve ser negligenciada. Relatamos dois casos de sarcoma pélvico: o primeiro imitando granuloma eosinofílico e o segundo imitando osteomielite. Neste último, também relatamos um achado atípico de sua história natural: uma resposta inicial ao antibiótico e ao tratamento anti-inflamatório. Em ambos os casos, destacamos a possibilidade de uma biópsia óssea percutânea inconclusiva e a importância da imunoquímica e da citogenética para o diagnóstico definitivo.

Application of fascial space priority approach for pelvic exenteration / 中华胃肠外科杂志

Locally advanced tumor with involvement of surrounding tissues and organs is a common situation in pelvic malignancies. Up to 10% of newly diagnosed rectal cancer cases infiltrate to adjacent tissues and organs. Satisfactory resection margins obtained by pelvic exenteration can achieve a 5-year survival rate similar to cases that without adjacent tissue invasion. The 5-year survival rate of patients with locally recurrent pelvic malignancies is almost zero if they are treated only with radiotherapy and chemotherapy. To obtain negative margins through pelvic exenteration is the only chance for a long-term survival of these patients. However, pelvic exenteration is a complicated procedure with higher morbidity and mortality. The development of fascia anatomy enables surgeons to have a deeper understanding and comprehensive application of pelvic fasciae. Meanwhile, the improvement of laparoscopic technology provides a clearer view for surgeons and enables the application of minimally invasive techniques in complex pelvic exenteration. The fascial space priority approach is based on the fascia anatomy of pelvis and giving priority to the separation of the pelvic avascular fascial spaces, which provides a reproducible surgical approach for complex pelvic exenteration.

Observational study on perioperative outcomes of pelvic exenteration / 中华胃肠外科杂志

Objective: To investigate the surgical indications and perioperative clinical outcomes of pelvic exenteration (PE) for locally advanced, recurrent pelvic malignancies and complex pelvic fistulas. Methods: This was a descriptive study.The indications for performing PE were: (1) locally advanced, recurrent pelvic malignancy or complex pelvic fistula diagnosed preoperatively by imaging and pathological examination of a biopsy; (2)preoperative agreement by a multi-disciplinary team that non-surgical and conventional surgical treatment had failed and PE was required; and (3) findings on intraoperative exploration confirming this conclusion.Contraindications to this surgical procedure comprised cardiac and respiratory dysfunction, poor nutritional status,and mental state too poor to tolerate the procedure.Clinical data of 141 patients who met the above criteria, had undergone PE in the Sixth Affiliated Hospital of Sun Yat-sen University from January 2018 to September 2022, had complete perioperative clinical data, and had given written informed consent to the procedure were collected,and the operation,relevant perioperative variables, postoperative pathological findings (curative resection), and early postoperative complications were analyzed. Results: Of the 141 included patients, 43 (30.5%) had primary malignancies, 61 (43.3%) recurrent malignancies, 28 (19.9%) complex fistulas after radical resection of malignancies,and nine (6.4%)complex fistulas caused by benign disease. There were 79 cases (56.0%) of gastrointestinal tumors, 30 cases (21.3%) of reproductive tumors, 16 cases (11.3%) of urinary tumors, and 7 cases (5.0%) of other tumors such mesenchymal tissue tumors. Among the 104 patients with primary and recurrent malignancies, 15 patients with severe complications of pelvic perineum of advanced tumors were planned to undergo palliative PE surgery for symptom relief after preoperative assessment of multidisciplinary team; the other 89 patients were evaluated for radical PE surgery. All surgeries were successfully completed. Total PE was performed on 73 patients (51.8%),anterior PE on 22 (15.6%),and posterior PE in 46 (32.6%). The median operative time was 576 (453,679) minutes, median intraoperative blood loss 500 (200, 1 200) ml, and median hospital stay 17 (13.0,30.5)days.There were no intraoperative deaths. Of the 89 patients evaluated for radical PE surgery, the radical R0 resection was achieved in 64 (71.9%) of them, R1 resection in 23 (25.8%), and R2 resection in two (2.2%). One or more postoperative complications occurred in 85 cases (60.3%), 32 (22.7%)of which were Clavien-Dindo grade III and above.One patient (0.7%)died during the perioperative period. Conclusion: PE is a valid option for treating locally advanced or recurrent pelvic malignancies and complex pelvic fistulas.

Recognition of empty pelvic syndrome and its prevention and treatment / 中华胃肠外科杂志

With the development of existing surgical techniques, equipment and treatment concepts, more and more medical centers begin to carry out extensive resection for recurrent pelvic malignant tumors or those with multivisceral invasion. Exenteration may facilitate curative resection and improve the outcome of the patients. Therefore, pelvic exenteration has gradually become the standard of care for locally advanced pelvic malignancies. At present, pelvic exenteration leads to high intraoperative and postoperative complications and mortality, and therefore compromise the safety and long-term quality of life. Cumulating evidences suggest remnant cavity after exenteration might trigger the pathophysiological process and cause downstream complications which can be defined as empty pelvis syndrome. The literature related to empty pelvic syndrome was summarized, the possible cause of empty pelvic syndrome was analyzed. After the pelvic exenteration, the closed pelvic residual cavity formed continuous negative pressure with the gradual absorption of air in the cavity, bacterial propagation, and accumulation of fluid, which had an impact on the distribution of organs in the abdominal and pelvic cavity. At the same time, whether physical processes also play a role in the occurrence of empty pelvic syndrome remains to be explored. It is concluded that the diagnosis is mainly based on the patient's medical history, clinical manifestations and radiological findings, and the history of pelvic exenteration is the most important indicator in the diagnosis. In terms of prevention measures, we should identify the high-risk groups of the occurrence of empty pelvic syndrome, and then take accurate and individualized preventive measures. Various new biomaterials have more advantages in preventive pelvic cavity filling than traditional human tissue filling. Mesentery plays an important role in the morphology, peristalsis and arrangement of the small intestine. More attention should be paid to reducing the ectopic placement of the small intestine into the pelvic cavity by protecting the mesentery structure and restoring or rebuilding the mesentery morphology. In terms of treatment measures, there is still a lack of standard treatment pathway for empty pelvic syndrome.

Angiomixoma agresivo pélvico: reporte de caso y revisión / Aggressive pelvic angiomyxoma: case report and revision / Angiomixoma agressivo: relato de caso e revisão

El angiomixoma agresivo pélvico es un tumor de partes blandas extremadamente raro, del cual se han reportado alrededor de 350 casos hasta la fecha. A continuación se reporta el caso clínico de una paciente que presentó dicho tumor y se describe el proceso diagnóstico y su tratamiento quirúrgico. Por otro lado, se realiza una breve revisión de la literatura disponible hasta el momento sobre el tema.

Aggressive pelvic angiomyxoma is an extremely rare soft tissue tumor, of which around 350 cases have been reported to date. This article aims at reporting the case of a patient presenting said tumor, as well as describing its diagnostic approach and its surgical treatment. On the other hand, it aims at briefly reviewing the available literature on the subject.

O angiomixoma pélvico agressivo é um tumor extremamente raro dos tecidos moles, tendo sido descritos até à data cerca de 350 casos. Relatamos o caso de uma doente que apresentava este tumor e descrevemos o processo de diagnóstico e tratamento cirúrgico. É também feita uma breve revisão da literatura disponível até à data sobre o assunto.

Fibromatosis mesentérica solitaria. Reporte de caso y revisión de la literatura / Sporadic mesenteric fibromatosis. A case report. Literature review / Fibromatose mesentérica solitária. Relato de caso. Revisão da literatura

Resumen: La fibromatosis mesentérica es un subtipo profundo de tumor desmoide (TD), un tumor benigno de origen fibroblástico localmente agresivo por su tendencia a infiltrar los tejidos adyacentes. Son raros, esporádicos y pueden asociarse con el síndrome de Gardner. El tratamiento de elección es la resección completa, evitando la recurrencia local. Comunicamos el caso clínico de una paciente con fibromatosis intrabdominal mesentérica única, bien circunscripta, que simulaba por la imagenología una masa de origen pelviano.

Summary: Mesenteric fibromatosis is a deep sub-type of desmoid tumors consisting of a benign tumor of fibroblastic origin which is locally aggressive given its tendency to infiltrate adjacent tissues. They are unusual and sporadic, and may be associated to Gardner's Syndrome. Complete resection is the treatment of choice, avoiding local recurrence. The study reports the clinical case of a patient with intra-abdominal sporadic mesenteric fibromatosis, well circumscribed that appeared to be a pelvic mass in MR imaging.

Resumo: A fibromatose mesentérica é um subtipo profundo de tumor desmóide (DT); é um tumor benigno de origem fibroblástica que é localmente agressivo devido à sua tendência a infiltrar tecidos adjacentes. São raros, esporádicos e podem estar associados à síndrome de Gardner. O tratamento de escolha é a ressecção completa, evitando recidiva local. Relatamos o caso clínico de uma paciente com fibromatose mesentérica intra-abdominal única e bem circunscrita que simulava uma massa de origem pélvica na imagem.

Therapeutics in radiation-induced proctopathy: a systematic review

Malignant neoplasms are increasingly prevalent in the daily clinical practice. Up to 61% of patients with pelvic malignancies undergo pelvic radiotherapy in different doses, which may cause intestinal damage, and the rectum is the segment most frequently affected due to its fixed position in the pelvis. Currently, there are several strategies to minimize the effects of radiation on the tissues surrounding the neoplastic site; despite those strategies, radiotherapy can still result in serious damage to organs and structures, and these injuries accompany patients throughout their lives. One of the most common damages resulting from pelvic radiotherapy is acute proctitis.The diagnosis is confirmed by visualizing the rectal mucosa through rigid or flexible rectosigmoidoscopy and colonoscopy. The objective of the present study was to review the forms of radiation-induced proctopathytherapy, and to evaluate the results of each method to propose a standardization for the treatment of this pathology. Despite the prevalence of radiation-induced proctopathy, there is no definitive standardized treatment strategy so far. The first approach can be tried with local agents, such as mesalazine and formalin. For refractory cases, control can usually be achieved with argon plasma coagulation, hyperbaric oxygen, and radiofrequency ablation therapies. Regarding the study of radiation-induced proctopathy, there is a lack of robust studies with large samples and standardized therapies to be compared. There is a lack of double-blinded, randomized controlled studies to determine a definitive standard treatment algorithm. (AU)

Tumor Fibroso Solitário Primário em Região Pélvica: Relato de Caso / Primary Solitary Fibrous Tumor in Pelvic Region: Case Report / Tumor Fibroso Solitario Primario en la Región Pélvica: Relato de Caso

Introdução: O tumor fibroso solitário é uma neoplasia mesenquimal rara, originada de células intersticiais dendríticas CD34 positivas e composta pela justaposição de células fusiformes. Os casos iniciais foram primariamente descritos na região torácica, e o principal sítio, a pleura visceral. Raramente são descritos casos de tumor fibroso solitário extrapleural em região pélvica, demonstrando a possibilidade de múltiplas sítios primários. Relato do caso: Paciente de 38 anos, sexo feminino, previamente saudável e assintomática, procurou orientação médica após uma ultrassonografia pélvica de rotina evidenciar uma imagem heterogênea na região anexial direita, paraovariana, sendo submetida à ressecção cirúrgica da lesão, com diagnóstico de tumor fibroso solitário confirmado por imuno-histoquímica. Discutem-se os aspectos do diagnóstico imuno-histoquímico e do tratamento cirúrgico. Conclusão: Os casos de tumor fibroso solitário em região pélvica podem ser descobertos por meio de ultrassonografia pélvica de rotina. O tratamento cirúrgico, com ressecção ampliada e margens negativas, deve ser o principal objetivo nos casos de tumor fibroso solitário em região pélvica. A obtenção de amostras para análise imuno- -histoquímica é recomendada, e a positividade para CD34 e STAT6 aponta o diagnóstico. Recidivas podem ocorrer em até uma década de seguimento, sendo recomendado período longo de acompanhamento pós-cirúrgico

Introduction: Solitary fibrous tumor is a rare mesenchymal neoplasm, originated from CD34-positive interstitial dendritic cells and composed by the juxtaposition of spindle cells. Initial cases were primarily described in the thoracic region, the main site being the visceral pleura. However, cases of solitary extra-pleural fibrous tumors have already been described in the pelvic region demonstrating the possibility of multiple primary sites. Case report: A 38-year-old female patient, previously healthy and asymptomatic, seeks medical advice after a routine pelvic ultrasound that showed a heterogeneous image in the right adnexal region, paraovarian, being submitted to surgical resection of the lesion with a diagnosis of solitary fibrous tumor confirmed by immunohistochemistry. Aspects of immunohistochemical diagnosis and surgical treatment were discussed. Conclusion: Cases of solitary fibrous tumor in the pelvic region can be discovered through routine pelvic ultrasound. Surgical treatment, with extended resection and negative margins, should be the main objective in cases of solitary fibrous tumor in the pelvic region. Obtaining samples for immunohistochemical analysis is recommended and positivity for CD34 and STAT6 discloses the diagnosis. Relapses can occur in up to a decade of follow-up, and a long period of post-surgical follow-up is recommended

Introducción: El tumor fibroso solitario es una neoplasia mesenquimatosa rara, originada a partir de células dendríticas intersticiales CD34 positivas y compuesta por la yuxtaposición de células fusiformes. Los casos iniciales se describieron principalmente en región torácica, siendo el sitio principal la pleura visceral. Sin embargo, ya se han descrito los casos de tumores fibrosos extrapleurales solitarios, como en la región pélvica. Relato del caso: Paciente femenina de 38 años, previamente sana y asintomática, acude al médico luego de una ecografía pélvica de rutina que mostró una imagen heterogénea en región anexial derecha, para ovárica. La paciente fue tratada con resección quirúrgica de la lesión y tuvo el diagnóstico de tumor fibroso solitario confirmado por inmunohistoquímica. Se discuten aspectos del diagnóstico inmunohistoquímico y del tratamiento quirúrgico. Conclusión: Los casos de tumor fibroso solitario en la región pélvica se pueden descubrir mediante una ecografía pélvica de rutina. El tratamiento quirúrgico, con resección ampliada y márgenes negativos, debe ser el principal objetivo en los casos de tumor fibroso solitario en la región pélvica. Se recomienda obtener muestras para análisis inmunohistoquímico y la positividad para CD34 y STAT6 apunta al diagnóstico. Las recaídas pueden ocurrir hasta en una década de seguimiento, y se recomienda un largo período de seguimiento posquirúrgico

Chronic radiation-induced rectal injury after adjuvant radiotherapy for pelvic malignant tumors: report based on a phase 3 randomized clinical trial / 中华胃肠外科杂志

Objective: Radiotherapy is one of the standard treatments for pelvic malignant tumors. However, researches associated with intestinal radiation injury and the quality of life (QoL) of patients receiving radiotherapy were lacking in the past. This study aims to analyze the occurrence of radiation-induced rectal injury after adjuvant radiotherapy for pelvic malignant tumors and call for more attention on this issne. Methods: A retrospectively observational study was conducted. Case data of cervical cancer patients from the database of STARS phase 3 randomized clinical trial (NCT00806117) in Sun Yat-sen University Cancer Center were analyzed. A total of 848 cervical cancer patients who received adjuvant radiation following hysterectomy and pelvic lymphadenectomy in Sun Yat-sen University Cancer Center from February 2008 to August 2015 were recruited. The pelvic radiation dosage was 1.8 Gy/day or 2.0 Gy/day, five times every week, and the total dosage was 40-50 Gy. Among 848 patients, 563 patients received radiation six weeks after surgery, of whom 282 received adjuvant radiation alone and 281 received concurrent chemoradiotherapy (weekly cisplatin); other 285 patients received sequential chemoradiotherapy (paclitaxel and cisplatin). Acute adverse events, chronic radiation damage of rectum, and QoL were collected and analyed. The digestive tract symptoms and QoL were evaluated based on EORTC QLQ-C30 questionnaires at one week after surgery (M0), during adjuvant therapy period (M1), and at 12 months and 24 months after the completion of treatments (M12 and M24), respectively. Higher scores in the functional catalog and overall quality of life indicated better quality of life, while higher scores in the symptom catalog indicated severe symptoms and worse QoL. Chronic radiation rectal injury was defined as digestive symptoms that were not improved within three months after radiotherapy. Grading standard of acute adverse events and chronic radiation rectal injury was according to the gastrointestinal part of National Cancer Institute Common Terminology Criteria for Adverse Events, version 4.0 (NCI-CTCAE Version 4.0). Results: The mean total radiation dosage of 848 patients was (47.8±4.6) Gy. During adjuvant therapy, the common symptoms of acute intestinal dysfunction were nausea (46.0%, 390/848), vomiting (33.8%, 287/848), constipation (16.3%, 138/848) and abdominal pain (10.3%, 87/848). At M12 and M24, the number of 0 QLQ-C30 questionnaires collected was 346 and 250, respectively. QLQ-C30 questionnaires showed that the scores of nausea or vomiting, appetite decrease, diarrhea, constipation, etc. were improved obviously at M12 or M24 compared with those at M0 or during M1 (all P<0.05). As the extension of the follow-up time, the score of the overall QoL of patients gradually increased [M0: 59.7 (0.0-100.0); M1: 63.1 (0.0-100.0); M12: 75.2 (0.0-100.0); M24: 94.1 (20.0-120.0); H=253.800, P<0.001]. Twelve months after the completion of treatments, the incidence of chronic radiation rectal injury was 9.8% (34/346), mainly presenting as abdominal pain, constipation, stool blood, diarrhea, mostly at level 1 to 2 toxicity (33/34, 97.1%). One patient (0.3%) developed frequent diarrhea (>8 times/d), which was level 3 toxicity. Twenty-four months after all treatments, the incidence of chronic radiation rectal injury was 9.6% (24/250), which was not decreased significantly compared to that in the previous period (χ(2)=0.008, P=0.927). The symotoms of one patient with level 3 toxicity was not relieved. Conclusions: The common symptoms of patients with pelvic maligant tumors during postoperative adjuvant radiotherapy include nausea, vomiting, constipation, abdominal pain and diarrhea. These symptoms are alleviated obviously at 12 and 24 months after adjuvant radiotherapy, and the QoL is significantly improved. However, a few patients may develop chronic radiation rectal injury which is not improved for years or even decades, and deserves attention in clinical practice.

Acute radiodermatitis in cancer patients: incidence and severity estimates / Radiodermatitis aguda en pacientes con cáncer: estimación de la incidencia y la severidad / Radiodermatite aguda em pacientes com câncer: estimativa de incidência e severidade

ABSTRACT Objective: To estimate the incidence and distribution of the degree of radiodermatitis in cancer patients submitted to radiotherapy in the regions of head and neck, breast, and pelvis. Method: Prospective, longitudinal, quantitative study conducted from March 15, 2016 to May 4, 2017 with patients observed for five weeks of treatment. The primary outcome was the proportion of grade 1 or higher radiodermatitis each week. The degree of radiodermatitis and mean time of occurrence for each patient were secondary outcomes. Results: This study included 112 patients. The incidence of radiodermatitis was 100% among those whose head and neck region was irradiated, followed by 98% for breast and 48% for the pelvic region. The degree of radiodermatitis varied with the irradiated site; the worst degrees were found in the head and neck region, followed by the breast. In the three groups, the mean time of the first occurrence of radiodermatitis was approximately eleven days. Conclusion: The incidence of radiodermatitis in the studied groups was high, which reinforces the need to perform the same evaluation nationwide. This would support creating and standardizing protocols and recommendations for a proper management of radiodermatitis, specially concerning its prevention.

RESUMEN Objetivo: Estimar la incidencia y la distribución del grado de radiodermatitis en pacientes con cáncer sometidos a radioterapia en las regiones de la cabeza y el cuello, la mama y la pelvis. Método: Estudio prospectivo, longitudinal y cuantitativo realizado entre el 15 de marzo de 2016 y el 4 de mayo de 2017 con pacientes observados durante cinco semanas de tratamiento. El resultado primario fue la proporción de radiodermatitis grado 1 o superior cada semana. El grado de radiodermatitis y el tiempo medio de la primera aparición en cada paciente fueron resultados secundarios. Resultados: Participaron 112 pacientes. La incidencia de radiodermatitis fue del 100% entre los que se irradiaron en la región de la cabeza y el cuello, seguida del 98% en la mama y el 48% en la pelvis. El grado de radiodermatitis variaba según el sitio de irradiación, con peores grados en la región de la cabeza y el cuello, seguida de la mama. En los tres grupos, el tiempo medio para la primera aparición de radiodermatitis fue de aproximadamente 11 días. Conclusión: La incidencia de la radiodermatitis fue elevada en los grupos estudiados, lo que reitera la necesidad de hacer la misma evaluación en todo el país. Esta evaluación fomentaría la creación y la uniformización de los protocolos y recomendaciones para el manejo adecuado de la radiodermatitis, especialmente en lo que respecta a su prevención.

RESUMO Objetivo: Estimar a incidência e a distribuição de graduação de radiodermatite em pacientes com câncer submetidos à radioterapia nas regiões de cabeça e pescoço, mama e pelve. Método: Estudo prospectivo, longitudinal, quantitativo, realizado durante o período de 15 de março de 2016 a 4 de maio de 2017 com pacientes observados ao longo de cinco semanas de tratamento. O desfecho primário foi proporção de radiodermatite grau 1 ou superior em cada semana. Graduação de radiodermatite e tempo médio para a primeira ocorrência em cada paciente foram desfechos secundários. Resultados: Participaram 112 pacientes. A incidência de radiodermatite foi de 100% entre os que irradiaram a região de cabeça e pescoço, seguida por 98% em mama e 48% na pelve. A graduação de radiodermatite variou de acordo com o local da irradiação, com piores graduações na região de cabeça e pescoço, seguida por mama. Nos três grupos, o tempo médio para a primeira ocorrência de radiodermatite foi de aproximadamente 11 dias. Conclusão: A incidência de radiodermatite foi alta nos grupos estudados, o que reitera a necessidade de se fazer a mesma avaliação em âmbito nacional. Essa avaliação incentivaria a criação e a uniformização de protocolos e recomendações para o manejo adequado da radiodermatite, principalmente no que concerne à sua prevenção.

Tailgut cyst adenocarcinoma

Tailgut cysts (TGCs) are rare congenital entities arising from remnants of the embryological postanal primitive gut. Malignancy in TGCs is rare, with the majority being adenocarcinomas and carcinoid tumors. A search of the published literature yielded only 27 cases of adenocarcinoma developing in TGCs. We described the case of a 54-year-old female who presented with complaints of pelvic and perineal pain of several weeks. After the initial work-up, a mass in the right presacral location was diagnosed. She underwent radical resection of the tumor, using a posterior approach. The lesion was removed en bloc with the middle rectum, coccyx, and sacrum (S4­S5). The histopathologic examination revealed an adenocarcinoma arising in a TGC, and the patient received adjuvant chemoradiotherapy. Our case underlines that diagnosing a TGC is difficult as it is a rare congenital lesion. Clinical examination may be challenging as TGCs present with various symptoms, which can mimic other commonly proctologic disorders. Patients should be referred to a tertiary center with experience in pelvic surgery and must be managed by a multidisciplinary approach to maximize successful treatment. The recommended treatment is surgical excision given the malignant potential of TGCs and their risk of causing local complications.

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.

Pleomorphic hyalinizing angiectatic tumor of the mesorectum: a rare case and review of literature

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare tumor of uncertain tissue origin. Although it has been classified as a benign tumor under the WHO classification, it is locally aggressive, and multiple recurrences have been reported. PHAT commonly involves the lower extremities; however, various unusual sites of origin have been reported. We present the case of a 30-year-old female with dysmenorrhea, who presented a presacral mass on imaging. The core biopsy confirmed the diagnosis of PHAT. She underwent laparotomy and excision. Histopathology confirmed the presence of a tumor comprised of aggregates of ectatic vessels with perivascular hyalinization. An immunohistochemical study showed diffuse CD34 positivity, but S100, MDM2, and smooth muscle actin negativity. After surgical procedures, the patient is disease free as at the 12-month follow-up. Only 120 cases have been published in the English literature to date. Our study is only the third case of PHAT arising from the pelvis to be reported. Though considered to be a rare condition, the diagnosis of PHAT should always be considered in the differential diagnosis of well-defined hypervascular soft tissue mass in the pelvis. The typical histopathological findings along with immunohistochemistry should clinch the diagnosis.

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.

Imaging description of extragenital müllerian adenosarcoma: A case report / Descrição da imagem do adenosarcoma mülleriano extragenital: relato de caso

Abstract Müllerian adenosarcoma is a very rare gynecological disease, comprising 5% of uterine sarcomas. Extragenital localizations are even rarer.We report a very interesting case of a 27-year-old woman complaining of pelvic pain, with a subsequent diagnosis of extragenital Müllerian adenosarcoma. This is the first case reported in the literature with a complete and wide imaging description. Even if rare, Müllerian adenosarcoma should be hypothesized in case of young female patients presenting with suspicious pelvic mass.

Resumo O adenosarcoma Mülleriano é uma doença ginecológica muito rara, compreendendo 5% dos sarcomas uterinos. Localizações extragenitais são ainda mais raras. Relatamos um caso muito interessante de uma mulher de 27 anos queixando-se de dor pélvica com diagnóstico subsequente de adenosarcoma Mülleriano extragenital. Este é o primeiro caso relatado na literatura com uma descrição completa e ampla de imagem. Mesmo que raro, o adenosarcoma Mülleriano deve ser hipotetizado no caso de pacientes jovens do sexo feminino com massa pélvica suspeita.

Bilateral Ureteral Obstruction Related to Pelvic Rhabdomyosarcoma Presenting with Acute Kidney Injury: A Case Report

Bilateral renal obstruction is a rare critical condition, requiring a prompt diagnostic approach and treatment to restore the renal function. The most commonly observed obstructive uropathy in children is congenital malformation, such as posterior urethral valves and bilateral ureteropelvic junction obstruction. Malignant pelvic masses obstructing the ureter are widely reported in adults but are rarely observed in children. The treatment of ureteral obstruction related to pelvic malignancy is a therapeutic challenge with a median survival duration of 3–7 months in adults; however, pediatric patients with pelvic malignancy leading to ureteral obstruction had better outcomes, with a reported 5-year mortality rate of 20%, than the adult patients. Here, we report a rare case of bilateral ureteral obstruction associated with pelvic rhabdomyosarcoma presenting with acute kidney injury treated by ureteral diversion with double J stent, and concommittent emergency hemodialysis, leading to restoration of good renal function. We suggest that bilateral ureteral obstruction should be released as soon as possible using surgical or interventional approach to minimize the obstruction period, and subsequential chemotherapy may contribute to improvement of survival and recovery of renal function.

Hydrogen-water ameliorates radiation-induced gastrointestinal toxicity via MyD88’s effects on the gut microbiota

Although radiation therapy is a cornerstone of modern management of malignancies, various side effects are inevitably linked to abdominal and pelvic cancer after radiotherapy. Radiation-mediated gastrointestinal (GI) toxicity impairs the life quality of cancer survivors and even shortens their lifespan. Hydrogen has been shown to protect against tissue injuries caused by oxidative stress and excessive inflammation, but its effect on radiation-induced intestinal injury was previously unknown. In the present study, we found that oral gavage with hydrogen-water increased the survival rate and body weight of mice exposed to total abdominal irradiation (TAI); oral gavage with hydrogen-water was also associated with an improvement in GI tract function and the epithelial integrity of the small intestine. Mechanistically, microarray analysis revealed that hydrogen-water administration upregulated miR-1968-5p levels, thus resulting in parallel downregulation of MyD88 expression in the small intestine after TAI exposure. Additionally, high-throughput sequencing showed that hydrogen-water oral gavage resulted in retention of the TAI-shifted intestinal bacterial composition in mice. Collectively, our findings suggested that hydrogen-water might be used as a potential therapeutic to alleviate intestinal injury induced by radiotherapy for abdominal and pelvic cancer in preclinical settings.