Special issue "The advance of solid tumor research in China": Presurgical therapy in the management of local retroperitoneal recurrence of renal cell carcinoma after radical nephrectomy.

Local retroperitoneal recurrence (RPR) after radical nephrectomy (RN) is rare in patients with renal cell carcinoma (RCC); however, it is associated with poor prognosis and lacks standard treatment. Our study aimed to assess oncological outcomes and prognostic factors of patients that underwent targeted therapy for RPR after RN, and to evaluate the role of presurgical targeted therapy in this context. This was a retrospective multicenter study of 85 patients with RPR treated with targeted therapy for RPR after RN (July 2008-October 2020). Clinical and pathological characteristics were reported using descriptive statistics. Cancer-specific survival (CSS) was examined using the Cox proportional hazards model. The median follow-up time was 50 months (95% confidence interval [CI]: 33.3-66.7) after the RPR diagnosis. The median CSS was 96 months in the presurgical targeted therapy followed by surgical resection group and 42 months (95% CI: 28.8-55.2) in the targeted therapy alone group (P = .0011). In multivariate analysis, International Metastatic RCC Database Consortium classification intermediate/poor risk, number of recurrence lesions and surgical resection were independent predictors of CSS. Presurgical targeted therapy may increase the feasibility of tumor resection for RPR after RN. Patients who underwent surgical resection following presurgical targeted therapy had better CSS than those treated with targeted therapy alone.

Robot-Assisted Laparoscopic Excision of Complicated Retroperitoneal Tumors with Four Arms Via Retroperitoneal Way: A Unique Minimal-Invasive Approach.

Background: Surgical management of complicated retroperitoneal mass is one of the most challenging urologic oncologic surgeries. This study aims to describe our technique and experience in dealing with retroperitoneal mass. Methods: Three patients with complicated retroperitoneal mass were treated with robot-assisted surgery with four arms through retroperitoneal approach. Surgical Procedure: Our standardized anatomic-based "kidney safe first, then mass resection" technique for robot-assisted complicated retroperitoneal mass resection focused on minimizing the chance of renal pedicle injury. Baseline demographics, pathology data, and latest follow-up outcome were obtained. Results: In this retrospectively reviewed case series, all 3 patients were successfully treated with robot-assisted surgery with four arms during retroperitoneal space. One patient received paravertebral mass resection 2 weeks after the robotic surgery. Mean data included operative time of 175 minutes, estimated blood loss was 133 mL, and hospital stay was 4 days. No complications occurred. Conclusions: Robot-assist surgery for complicated retroperitoneal mass with four arms is a safe and feasible way. Patient Summary: Mini-invasive treatment for retroperitoneal mass with robotic four arms through retroperitoneal approach is a feasible way. The approach reduces interruption of intracorporeal structure and organs. And patients could benefit from the retroperitoneal approach with a quicker recovery.

Masa retroperitoneal gigante en el tumor periférico de la vaina neural asociado a neurofibromatosis tipo 1. Reporte de un caso y revisión de la literatura / Giant retroperitoneal mass in peripheral neural sheath tumor associated with neurofibromatosis type 1. Case report and review of the literature

Los tumores malignos de vaina neural periférica (MPNST) se observan con mayor frecuencia en pacientes con neurofibromatosis tipo 1 (NF-1), presentando un crecimiento rápido y unas tasas de supervivencia a los 5 años alrededor del 33%. Presentamos el caso de un varón de 17 años afecto de NF-1 con un MPNST retroperitoneal gigante sometido a tratamiento quirúrgico radical, con recidiva tumoral precoz

Malignant peripheral nerve sheath tumors (MPNST) are most frequently in patients with neurofibromatosis type 1 (NF-1), with a rapid growth and 5-years survival rates around 33%. We present the case of a 17 years old male with NF-1 and a giant retroperi-toneal MPNST who undergoes a radical surgical treatment, with early tumor recurrence

[Surgical treatment of neurofibromatosis type I followed by retroperitoneal tumor (in Russian only)]. / Khirurgicheskoe lechenie neirofibromatoza I tipa, assotsiirovannogo s opukholevym porazheniem zabriushinnogo prostranstva.

AIM: To determine the optimal surgical approach in patients with abdominal and retroperitoneal manifestations of Recklinghausen's disease. MATERIAL AND METHODS: There were 4 patients (3 women and 1 man) with neurofibromatosis type I who were treated at Vishnevsky National Medical Research Center of Surgery. RESULTS: There were 3 robot-assisted procedures: excision of retroperitoneal tumors (plexiform neurofibroma and schwannoma) in 2 cases and right adrenal pheochromocytoma in 1 patient. We also included 1 clinical case of conventional surgery for neurofibromatosis type I followed by multiple gastrointestinal stromal duodenal and intestinal tumors. In one case, postoperative period was complicated by fluid accumulation in the bed of previously removed tumor that required US-assisted drainage. Postoperative period was uneventful in other cases. CONCLUSION: Robot-assisted surgery is safe and effective in patients with Recklinghausen's disease followed by single abdominal and retroperitoneal tumors. It is more expedient to choose conventional technique for multiple tumors located in different parts of retroperitoneal space or abdominal cavity.

Retroperitoneal low grade endometrial stromal sarcoma with florid endometrioid glandular differentiation: Cytologic-histologic correlation and differential diagnosis.

Low grade endometrial stromal sarcoma (LGESS) is a rare neoplasm that typically arises in the uterine corpus and accounts for less than 1% of uterine sarcomas. Infrequently, extra-uterine LGESS can occur. Histologically, LGESS is characterized by a monotonous population of cells that resemble the proliferative phase of endometrial stroma and in their classic form they exhibit tongue-like growth pattern of infiltration and/or lymphovascular invasion. Infrequently LGESS can demonstrate various morphologic differentiation patterns, including endometrioid-type glands. We report the first fine needle aspiration (FNA) case of a periduodenal mass that was incidentally discovered on Computed Tomography (CT) scan of a 60-year-old female. The cytomorphologic and histologic findings and the immunohistochemical staining were consistent with a LGESS with endometrioid glandular differentiation. We are presenting the correlation between the cytologic, radiologic and pathologic features.

Schwannoma de retroperitoneo asociado a Neurofibromatosis tipo I / Retroperitoneal Schannoma associated to type I neurofibromatosis

Los Schwannomas son tumores de origen neural, corresponden al 1 por ciento de los tumores de retroperitoneo. En la mayoría de las ocasiones, son tumores de comportamiento benigno, encontrando que solo del 5 - 18 por ciento son malignos y aún menos frecuente es que sean de alto grado en su histología. Se trata de una paciente de 46 años de edad sin antecedentes crónico degenerativos. Con diagnóstico conocido de neurofibromatosis tipo I. Inició su padecimiento actual tres años previos a su ingreso en 2013, al presentar artralgia de rodilla derecha la cual se asocia a mialgias de forma ipsilateral. Se le solicitó como apoyo diagnostico una resonancia magnética de columna dorsal y lumbar, en la que se reporta una tumoración en la región del retroperitoneo de lado derecho con medidas de 10 x 17 cm con aparente origen espinal en L2 y L3. A pesar de ser masas de origen neural benignas la mayoría de las veces, no es infrecuente su malignización encontrando una cavidad con estructuras vecinas tomada por el tumor. En estos casos, es de vital importancia el manejo preoperatorio de manera multidisciplinaria tanto en la preparación preoperatoria, así como durante el procedimiento quirúrgico(AU)

Schwannomas are tumors of neural origin and account for 1 percent of retroperitoneal tumors. In many times, they are benign tumors and just 5 to 8 percent are malignant; high degree is even less frequent in its histology. This is a male patient aged 46 years with no history of chronic degenerative problems and diagnosis of type I neurofibromatosis. The disease appeared three years before his admission to hospital in 2013 because of right knee arthralgia associated to myalgias on the same side. Diagnostic support was requested in terms of performance of magnetic resonance of dorsal and lumbar spine; this test reported the presence of a tumor in the right side of the retroperitoneal region, it measured 10 x 17 cm with apparent spinal origin in L2 and L3. Although they are often masses of benign neural origin, their malignization is not rare. There was a cavity with adjoining structures affected by the tumor. In this type of cases, the preoperative management with multidisciplinary involvement both in the preoperative preparation and during the surgical procedure is of vital importance(AU)

Schwannoma de retroperitoneo asociado a Neurofibromatosis tipo I / Retroperitoneal Schannoma associated to type I neurofibromatosis

RESUMEN Los Schwannomas son tumores de origen neural, corresponden al 1 por ciento de los tumores de retroperitoneo. En la mayoría de las ocasiones, son tumores de comportamiento benigno, encontrando que solo del 5 - 18 por ciento son malignos y aún menos frecuente es que sean de alto grado en su histología. Se trata de una paciente de 46 años de edad sin antecedentes crónico degenerativos. Con diagnóstico conocido de neurofibromatosis tipo I. Inició su padecimiento actual tres años previos a su ingreso en 2013, al presentar artralgia de rodilla derecha la cual se asocia a mialgias de forma ipsilateral. Se le solicitó como apoyo diagnostico una resonancia magnética de columna dorsal y lumbar, en la que se reporta una tumoración en la región del retroperitoneo de lado derecho con medidas de 10 x 17 cm con aparente origen espinal en L2 y L3. A pesar de ser masas de origen neural benignas la mayoría de las veces, no es infrecuente su malignización encontrando una cavidad con estructuras vecinas tomada por el tumor. En estos casos, es de vital importancia el manejo preoperatorio de manera multidisciplinaria tanto en la preparación preoperatoria, así como durante el procedimiento quirúrgico(AU)

ABSTRACT Schwannomas are tumors of neural origin and account for 1 percent of retroperitoneal tumors. In many times, they are benign tumors and just 5 to 8 percent are malignant; high degree is even less frequent in its histology. This is a male patient aged 46 years with no history of chronic degenerative problems and diagnosis of type I neurofibromatosis. The disease appeared three years before his admission to hospital in 2013 because of right knee arthralgia associated to myalgias on the same side. Diagnostic support was requested in terms of performance of magnetic resonance of dorsal and lumbar spine; this test reported the presence of a tumor in the right side of the retroperitoneal region, it measured 10 x 17 cm with apparent spinal origin in L2 and L3. Although they are often masses of benign neural origin, their malignization is not rare. There was a cavity with adjoining structures affected by the tumor. In this type of cases, the preoperative management with multidisciplinary involvement both in the preoperative preparation and during the surgical procedure is of vital importance(AU)

[Intraperitoneal cystic lymphangioma and Crohn's disease: an exceptional association]. / Kystique intra-péritonéal et maladie de Crohn: à propos d'une association exceptionnelle.

Cystic lymphangioma is a rare benign malformative tumor of the lymphatic vessels which may occur in various locations. Intra-abdominal cystic lymphangioma is less frequent than cervicoaxillary cystic lymphangioma. Clinical presentation is polymorphic. Diagnosis is based on imaging data but it requires histological confirmation. Surgery is the gold standard treatment. We here report a rare case of acquired intraperitoneal cystic lymphangioma secondary to subtotal colectomy in a female patient with severe evolutive Crohn's disease treated with anti-TNF alpha. The patient presented with irreducible right painful paramedian mass with no impulse on coughing, suggesting the diagnosis of strangulated eventration within a surgical scar from midline laparotomy. She underwent emergency surgery. Surgical exploration showed multi-cystic intraperitoneal mass protrunding through the right paramedian eventration. Anatomo-pathological examination helped to confirm the diagnosis of cystic lymphangioma. Postoperatively, the mass was punctured twice to evacuate the fluid, because of incomplete surgical resection. This is the first reported case of cystic lymphangioma in a patient under anti-TNF alpha. It could be caused by disruption of the immune system and more specifically of the lymphocyte population. This association has not hitherto been established and experimental studies are necessary to accept or refuse this hypothesis.

Para-ovarian adrenal rest tumors: gynecologic manifestations of untreated congenital adrenal hyperplasia.

Congenital adrenal hyperplasia (CAH) is an inherited disorder of adrenal steroidogenesis often diagnosed in infancy. Gynecologists may encounter adult patients with CAH due to the clinical effects of increased androgens, e.g. hirsutism, clitoromegaly, oligomenorrhea, or, rarely, pelvic masses. This case report reviews the association of para-ovarian adrenal rest tumors with CAH, and the role of gynecologists in their evaluation and treatment. A 23-year-old woman with CAH (21-hydroxyase deficiency) untreated for the past 5 years presented with a pelvic mass and elevated serum testosterone (1433 ng/dL) and plasma ACTH (1117 pg/mL). Intraoperative findings revealed multiple retroperitoneal masses. Final pathology demonstrated adrenal rest tissue. Para-ovarian and ovarian adrenal rest tumors may present as a rare gynecologic manifestation in patients with untreated CAH.

Pencil beam scanning proton therapy for treatment of the retroperitoneum after nephrectomy for Wilms tumor: A dosimetric comparison study.

BACKGROUND: Multimodality treatment for patients with Wilms tumor has improved patient survival, but is associated with acute and long-term toxicity, partially due to irradiation. Proton therapy using pencil beam scanning (PBS) is a promising technique to reduce dose to organs at risk (OAR). In this study, we evaluate PBS plans for postoperative irradiation in patients with Wilms tumor. PROCEDURE: Patients were treated with anterior-posterior-posterior-anterior (AP-PA) photon fields encompassing the preoperative tumor volume. Patients requiring whole lung irradiation were treated with AP-PA photon fields covering the bilateral lungs. Prescription doses were generally 1,080 and 1,200 cGy, respectively. Flank PBS plans encompassing the ipsilateral retroperitoneum and para-arotic nodes were generated for dosimetric evaluation. RESULTS: Treatment records and comparison plans of 11 patients were reviewed. Mean dose and median dose to 50% or more of the contralateral kidney (D50) were 135 cGy and 139 cGy with photons and 52 cGy relative biological effectiveness (RBE) (P = 0.009) and 5 cGy RBE (P = 0.000001) with PBS. Mean dose and median D50 to bowel was 639 cGy and 979 cGy with photons and 379 cGy RBE (P = 0.001) and 47 cGy RBE (P = 0.004) with PBS. Mean dose and median D50 to the liver were 755 cGy and 1,013 cGy with photons and 411 cGy RBE (P = 0.02) and 132 cGy RBE (P = 0.02) with PBS. For patients with right-sided tumors, mean liver dose following sequential whole lung irradiation was 1,252 cGy with photons and 845 cGy RBE (P = 0.04) with PBS. DISCUSSIONS: PBS proton therapy is a feasible method for irradiating the retroperitoneum and provides significant sparing of dose to critical OAR. This may translate to improved long-term health outcomes for patients and warrants further clinical investigation.

Post-chemotherapy retroperitoneal teratoma in nonseminomatous germ cell tumors: Do predictive factors exist? Results from a national multicenter study.

BACKGROUND AND OBJECTIVES: To identify predictive preoperative factors of the presence of teratoma in retroperitoneal lymph node dissection specimens. METHODS: We performed a 20 years multicenter retrospective analysis of all patients who underwent retroperitoneal lymph node dissection for residual masses after chemotherapy (PC-RPLND). Patients had undergone PC-RPLND after chemotherapy for advanced testicular cancer. The histologic components of the primary tumor were compared with those of the residual masses using logistic regression. RESULTS: A total of 469 NSGCT patients underwent PC-RPLND (complete data available for 211). By PC-RPLND, necrosis was found in 84 cases, teratoma in 102 cases, and viable tumor in 25 cases. The univariate and multivariate analyses showed that teratoma (P = 0.001 and P = 0.002, respectively) and yolk sac tumor (P = 0.009 and P = 0.035, respectively) in orchiectomy specimens were statistically significant predictors of the presence of teratoma in retroperitoneal lymph nodes. CONCLUSIONS: PC-RPLND is the standard treatment for any supracentimetric residual lesion. This procedure is associated with a high morbidity, and almost half patients are overtreated. The presence of teratoma and yolk sac tumor in the orchiectomy specimen were independent significant predictors of teratoma in retroperitoneal masses. J. Surg. Oncol. 2016;114:992-996. © 2016 Wiley Periodicals, Inc.

Cancer Screening of Renal Transplant Patients Undergoing Long-Term Immunosuppressive Therapy.

OBJECTIVE: With this study we aimed to research the effects of immunosuppressive drugs, their cumulative doses, and viral infections on development of malign tumors in patients who have undergone treatment for 5 years. METHODS: We examined 100 patients who underwent renal transplantation from 2004 to 2009. Patients had mycophenolate mofetil and steroid in addition to cyclosporine, sirolimus, or tacrolimus as immunosuppressive treatment. For malignancy screening, physical examination, radiologic and endoscopic screening were done, and immunosuppressive drugs and their cumulative doses, age, sex, body mass index (BMI), dialysis history, and viral infection history were investigated. RESULTS: The mean age of patients was 42.03 ± 11.30 years. There were 1 colon cancer patient, 1 retroperitoneal liposarcoma, 1 renal oncocytoma, 3 Kaposi sarcoma patients treated with cyclosporine; in those treated with Tac there were 1 basal cell carcinoma, 1 Kaposi sarcoma, 2 thyroid carcinoma, 1 breast carcinoma, 1 bladder carcinoma, 1 renal cell carcinoma, and 1 colon carcinoma patients. The mean age of patients having carcinoma was statistically significant compared with those without cancer (P < .01). The prednisolone cumulative dose was significantly higher in carcinoma patients than in patients without carcinoma (P < .01). RESULTS: The use of long-term chronic immunosuppressive therapy may increase the development of cancer. The risk of carcinoma increases with increasing drug dose and time period of the immunosuppressive drug. There was not a negative effect on cancer prevalence in patients with cyclosporine or tacrolimus. But the cumulative dose of steroids significantly increased malignancy occurence.

From observation to aetiology: a case report of a twin fetus-in-fetu and a revisit of the known rarity.

A baby girl presented with an antenatal diagnosis of a retroperitoneal tumour. Postnatal imaging suggested that this mass contained two fetiform structures with spine and long bone formation. This teratomatous mass was completely excised at 3 weeks of age. Histology was consistent with twin fetuses-in-fetu, revealing two fetiform masses each with an umbilical cord connecting to a common placenta-like mass. Despite a difference in the weight of the twin fetuses-in-fetu, the level of organogenesis was identical and corresponded to fetuses of 10 weeks of gestation. Each mass had four limbs, intact skin, rib cage, intestines, anus, ambiguous genitalia, primitive brain tissue and a spine with ganglion cells in the cord. Although considered a mature teratoma in the current World Health Organization classification, the theory of formation from multiple pregnancies has been commonly implied in more recent literature. The true aetiology of this rare condition remains unclear.

Klippel-Trenaunay syndrome presenting with acanthocytosis and splenic and retroperitoneal lymphangioma: a case report.

INTRODUCTION: Klippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by bone and soft tissue hypertrophy, extensive hemangioma and venous abnormalities. We report the case of a patient with two additional rare clinical manifestations in the background of Klippel-Trenaunay syndrome, namely, acanthocytosis and splenic and retroperitoneal lymphangioma. CASE PRESENTATION: A 24-year-old Sri Lankan man from North Central Province in Sri Lanka presented to our general medical unit with symptomatic anaemia. He had been diagnosed with Klippel-Trenaunay syndrome at the age of six years, with hemihypertrophy of his right lower limb and strawberry naevi over both lower limbs. His blood film results were positive for acanthocytes, which accounted for more than 20% of the red blood cell population. He was also found to have extensive splenic lymphangiomas and a large retroperitoneal lymphangioma encasing the mesentric vessels in the right para-aortic region. An extensive battery of tests to identify a secondary cause for the acanthocytosis failed to show any positive results. CONCLUSIONS: Retroperitoneal lymphangioma has been reported in association with Klippel-Trenaunay syndrome once before, but an association with acanthocytosis has never been reported. Given the rarity of all three conditions this is not surprising. The cause of acanthocytosis in this setting is currently unresolved. It is plausible that this may be a primary association with Klippel-Trenaunay syndrome, as an alternative aetiology was not found.

Primary retroperitoneal Müllerian adenocarcinoma arising from endometriosis.

Primary retroperitoneal Müllerian adenocarcinoma (PRMA) is an extremely rare tumor and the cause remains unknown. We report a case of PRMA arising from endometriosis. A 52-year-old woman with a history of malignant lymphoma underwent a follow-up computed tomography scan, which revealed a retroperitoneal tumor. Immunohistochemical analysis of tumor resected during laparoscopic surgery showed adenocarcinoma positive for cytokeratin 7 and negative for cytokeratin 20. The patient had undergone hysterectomy and bilateral salpingo-oophorectomy 14 years ago for myoma uteri and endometrial cysts and was treated with estrogen-replacement therapy. The size of the tumor increased and laparotomy was performed. Histopathological examination showed adenocarcinoma resembling endometrial adenocarcinoma, which stained positive for cancer antigen 125, cancer antigen 19-9, estrogen receptor, and progesterone receptor immunohistochemically. The focus of the endometriosis was found at the edge of the tumor, and the stromal cells around the tumor cells were CD10 positive. The patient was diagnosed as having PRMA arising from endometriosis, and treated with adjuvant chemotherapy.

Castleman's disease. A rare cause of isolated retroperitoneal mass.

OBJECTIVES: We describe a case of hyaline vascular type Castleman 's disease with unifocal presentation as retroperitoneal mass. We describe the pathological and radiological findings and present a bibliographic review. METHODS: Castleman's disease is a rare benign disease of unknown etiology characterized by a lymphoproliferative disorder. Two clinical types have been described: localized and multicentric. Isolated retroperitoneal involvement is uncommon. RESULTS: 39-year-old female who complained of back pain. Abdominal CT scan identified a 5 cm retroperitoneal interaortocaval mass. Complete laparoscopic excision was performed. Pathological study showed localized angiofollicular hyperplasia (hyaline vascular type Castleman's disease). CONCLUSIONS: The presentation of Castleman's disease as isolated retroperitonealmass is quite rare and should be distinguished from other retroperitoneal lesions of malignant character. The treatment of choice is surgery, providing a definitive and curative diagnosis. Operative biopsy may be useful.