Pelvic Neuroblastoma of the Pediatric Prostate.

Neuroblastoma accounts for a significant portion of childhood tumors and can present in a variety of ways. Pelvic neuroblastoma has been reported but few cases exist of neuroblastoma invading or originating from the bladder or prostate. We present a 4-year-old patient with pelvic neuroblastoma arising from the prostate and describe the medical and surgical management of this challenging case. While pelvic neuroblastoma may have an improved prognosis, this case demonstrates the challenging surgical decisions that accompany these patients to maintain quality of life while balancing oncologic efficacy of treatment.

[Fast track recovery protocols for extended pelvic surgery]. / Protokoly uskorennogo vyzdorovleniya pri rasshirenno-kombinirovannykh operatsiyakh na organakh malogo taza.

OBJECTIVE: To compare the immediate results of extended pelvic surgery before and after introduction of standardized fast track surgery (FTS) protocol into routine clinical practice. MATERIAL AND METHODS: The study included 111 patients with pelvic tumors who underwent extended pelvic surgery. The control group included 59 patients whose perioperative management implied traditional approaches (2018-2019), the main group - 52 patients with FTS protocol (2020-2021). Age, BMI and ECOG status were similar. In the main group, females (90.4% vs. 74.6%; p=0.046), patients with recurrent (46.2% vs. 22.0%; p=0.009) and complicated tumors (26.9% vs. 11.9%; p=0.054) prevailed. Obstructive resection without anastomosis was less common in the main group (28.8% vs. 47.5%; p=0.068). RESULTS: Surgery time was higher (319±125 min vs. 236±79 min, p<0.001) in the main group, but blood loss (238±154 ml vs. 282±150 ml, p=0.029) and incidence of blood transfusions (23.1% vs. 42.4%, p=0.043) were lower. Moreover, complications (36.6% vs. 54.3%; p=0.086), mild complications (Clavien-Dindo class I-II) (11.6% vs. 28.8%; p=0.034) and local infectious complications (19.2% vs. 42.4%; p=0.009) were less common in the main group. Two patients died in the control group due to sepsis following colonic anastomosis and bladder suture failure, respectively. Postoperative hospital-stay was similar (14±9.1 days vs. 14.4±9 days; p=0.89). CONCLUSION: FTS protocol is possible and safe in patients with locally advanced and recurrent malignant pelvic tumors. This approach reduces blood loss, the number of blood transfusions and risk of postoperative infections.

Extragonadal Perirectal Mature Cystic Teratoma in the Adult Male.

Background: Extragonadal abdominopelvic teratomas in adults are extremely rare, and those in males are exceedingly rare. These masses are most commonly found incidentally and require surgical excision for diagnostic confirmation after a thorough workup. Case Presentation: This is a case report of a 49-year-old male who presented to a urology office with symptoms of hematuria, incidentally, found to have a pelvic mass on computed tomography urogram prompting colorectal surgical evaluation and subsequent laparoscopic complete excision. The clinical presentation, radiographic findings, and histopathological findings are described along with a literature review of extragonadal abdominopelvic mature cystic teratoma, also referred to as a sacrococcygeal teratoma. Discussion: A broad differential diagnosis was generated for this patient with a pelvic mass after complete work-up, consisting of a dermoid or epidermoid cyst, liposarcoma, or sacrococcygeal teratoma. Radiological features can aid in the diagnostic confusion that may present in the adult patient. Conclusion: Albeit rare in the male and adult population, sacrococcygeal teratoma is a plausible differential diagnosis for a pelvic mass. Underrepresented in the literature in regard to guidelines on management, complete surgical excision is the gold standard, with laparoscopy being a reasonable approach.

Differentiating renal pelvic cancer from renal cell carcinoma with 18-fluorodeoxyglucose positron emission tomography-computed tomography.

BACKGROUND: The differential diagnosis of pelvis renalis cancer (PRC) from renal cell cancer (RCC) is difficult. Because of that, in this study, we compared the standardized uptake value (SUV) with positron emission tomography-computed tomography (PET-CT) of the RCC and PRC. METHODS: Twenty-one patients (12 males, 9 females; age range: 33-74 years; mean age ± standard deviation [SD]: 57.14 ± 17.6) with suspected primary renal cell cancer as Group 1 and 8 patients (6 male, 2 female; age range, 61-81; mean age ± SD, 71.5 ± 5.65) with suspected renal pelvis cancer as Group 2 detected by conventional imaging techniques (CT, magnetic resonance [MR] imaging, ultrasound, intravenous urogram, CT urography, MR urography) underwent fluorine-18-fluorodeoxyglucose (18F-FDG) PET/CT imaging between August 2010 and October 2012. RESULTS: Mean age is 57.14 (33-74) years in Group 1 and 71.5 (61-81) years in Group 2, respectively. The mean maximum SUV (SUVmax) value was 4.6 ± 2.1 in RCC group and 16.6 ± 6.9 in PRC group. At the 18-FDG PET/CT scanning, SUVmax value higher in patients with PRC than in the patients with RCC. It was statistically different (P < 0.001). CONCLUSION: We suggested that PET/CT can be used for the differential diagnosis of renal pelvis tumor and RCC. However, further studies with larger patient number are needed to confirm our suggestion. To clarify the mechanisms of underlying these differences, molecular advanced molecular studies are needed.

Atypical presentation of intravascular leiomyomatosis mimicking advanced uterine sarcoma: modified laterally extended endopelvic resection with preservation of pelvic neural structures.

Intravascular leiomyomatosis is a rare, benign tumour of myometrial smooth muscle. Despite being non-invasive, these tumours can proliferate aggressively within vascular structures including pelvic vessels, the vena cava and the heart. We discuss a 77-year-old woman presenting with a 9 cm uterine mass extending into the right adnexa and ovarian vein. Following hysteroscopic biopsy, palliative radical surgical resection was performed for suspected stage IV leiomyosarcoma. Tumour extension into the pelvic sidewall and obturator fossa indicated a modified laterally extended endopelvic resection combined with skeletonisation and preservation of the pelvic neurovasculature, ultimately providing a curative procedure with minimal functional neurological morbidity. We present this unusual case to assist in the development of a consensus for optimal case management where formal guidelines are not yet available. We summarise current understanding of intravascular leiomyomatosis and highlight the value of advanced surgical techniques using knowledge of complex ontogenetic and pelvic neuroanatomy in its management.

Evaluating the effects of metal artifacts on dose distribution of the pelvic region.

AIM OF THE STUDY: Some cancerous patients have hip prosthesis of metal elements when they undergo radiation therapy. Metal implants are a cause of metal artifacts in computed tomography (CT) images due to their higher density compared to normal tissues. The aim of this study is to evaluate the quantitative effects of metal artifacts on dose distribution of the pelvic region. MATERIALS AND METHODS: Seven patients with metal implants in the pelvic region were scanned and CT images were exported to the Monaco treatment planning system. Based on the diagnosis of each patient, three-dimensional plans were implemented on CT images and dose distributions were extracted. At the next step, metal artifacts were contoured and electron densities of these new structures were modified to the extent of soft tissue. Finally, dose distributions and the differences were investigated by VeriSoft software. RESULTS: The results of this study showed that if the electron density to metal artifacts is not assigned properly, it will increase the calculated monitor units (MUs) by almost 3.78 MUs/fraction which will significantly affect total dose distribution of treatment. CONCLUSION: For the precise implementation of the treatment and in order to minimize the systematic errors related to the calculated MUs, necessary corrections on the electron density of metal artifacts should be considered before the treatment planning. The issue will be more critical in advanced treatment modalities where dose escalation is needed.

Intraabdominal Schwannomas Single-center experience.

OBJECTIVE: Intraabdominal schwannomas are rare benign tumors. In this study, we aimed to present our clinical experience in patients with intrabdominally located Schwannoma. MATERIAL-METHOD: Patients who received the diagnosis of intrabdominal schwannoma between 2011-2019 were retrospectively examined. Demographic and clinical characteristics, treatment methods, short- and long-term results and immunohistochemical characteristics of the patients were analyzed. RESULTS: A total of 7 patients were included in the study. Four patients were female and three were male. The mean age was 51.5 (31-63) years. The most common clinical presentation was abdominal pain (57.1%). Tumor location was stomach (n=2), pelvic region (n=2), rectum (n=1), retropancreas (n=1), and left juxtadrenal space (n=1). Postoperative wound infection developed in one patient and pancreatic fistula complication was seen in one patient. Re-admissions to the hospital were due to anemia and pleural effusion in two patients. The mean tumor diameter was 6 cm (0.3-13 cm). All patients were S 100 strongly positive Mitoses / 50 HPFs (high power field), <2 Ki67 <3%. The mean follow- up period was 60 months. Currently, 5 patients are being followed without disease, 1 patient survives despite recurrence and 1 patient has died due to non-cancer reasons. CONCLUSION: Intrabdominal schwannomas are rare tumors which most commonly exhibit gastrointestinal involvement. Since these tumors are mostly benign, the long-term prognosis of patients is good. Schwannoma should be kept in mind in the differential diagnosis of intrabdominal masses. Radical resections with high morbidity and mortality should be avoided if preoperative diagnosis is made. KEY WORDS: Abdominal tumor, Mesenchymal tumor, Nerve sheath tumor, Schwannoma.

[Extramammary myofibroblastoma affecting the pelvic region: a case report]. / Myofibroblastome extramammaire de localisation pelvienne: à propos d'un cas.

Mammary myofibroblastoma is a rare soft-tissue tumor. Extramammary myofibroblastomas are particularly rare. We here report the case of a 78-year-old man presenting with pelvic pain relieved by defecation or urination. Rectal examination showed a mass in front of the anterior rectal wall. The magnetic resonance imaging (MRI) showed a well-circumscribed and heterogeneous mass measuring 10 x 6 x 8cm located behind the bladder which was pushed forward in front of the rectosigmoid. Immunohistochemical analyses showed diffuse co-expression on CD34 cells and desmin, Rb expression on most cells, oestrogen receptor expression, intense and diffuse P16 expression and a ki67 proliferation index of 25%. The patient had no recurrence 8 months after radiotherapy followed by surgery. Breast myofibroblastoma is a rare and benign tumor. Recurrence is hardly observed after local treatment. This study highlights the supporting role of radiotherapy in the efficacy of surgery.

Patterns of recurrence in women with advanced and recurrent epithelial ovarian cancer treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

OBJECTIVE(S): To identify recurrence patterns and outcomes in women with advanced or recurrent epithelial ovarian cancer (EOC) after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). METHODS: This is an IRB-approved single-institution cohort study of women who underwent CRS+HIPEC for advanced or recurrent EOC followed in a prospective registry from 1/12/2014-3/1/2020. Recurrence locations were defined as pelvic, upper abdominal (UA) and/or extra-peritoneal (EP). Univariate analysis assessed associations between recurrence location, progression-free survival (PFS), and overall survival (OS). RESULTS: In total, 92 women with EOC underwent interval (56.5%; n=52) or recurrent CRS+HIPEC (43.5%; n=40). For interval CRS+HIPEC, recurrence locations were pelvic in 50.0% (n=15), UA in 23.3% (n=7) and EP in 56.7% (n=17); 40.0% (n=12) were EP alone. Similarly, for recurrent CRS+HIPEC, recurrence locations were pelvic (22.5%, n=9), UA (5.0%, n=2) and EP (60.0%, n=24); 66.7% (n=20) were EP alone. For both interval and recurrent CRS+HIPEC, median PFS was 10.5 vs. 13.0 months for pelvic and UA vs. EP only recurrences (p=0.02). Similarly, median OS was 29.2 months for pelvic and UA and not reached for EP only (p=0.05). For interval CRS+HIPEC, there was no difference in median PFS (10.6 vs. 11.7 months, p=0.68) and OS (27.1 vs. 24.8 months, p=0.96) for pelvic and UA vs EP alone. However, for recurrent CRS+HIPEC, pelvic and UA sites of recurrence were associated with reduced PFS (10.0 vs. 18.1 months, p=0.03) and OS (33.6 months vs. not reached, p=0.02) vs. EP only. CONCLUSIONS: In women with advanced or recurrent EOC undergoing CRS+HIPEC, one-half of patients experience their first recurrence outside of the peritoneal cavity. Providers must be aware of the risk of EP failure in patients treated with CRS+HIPEC.

pT3 subclassification of renal pelvic cancer considering the tumor location improves the patients' prognostic accuracy.

Whether pT3 urothelial carcinoma of the renal pelvis (UCRP) and urothelial carcinoma of the ureter (UCU) have the same prognosis is controversial, this study compared the prognosis of pT3 UCRP with that of pT3 UCU. We retrospectively evaluated 954 patients who underwent nephroureterectomy at our institutions between January 1983 and December 2017. All surgical specimens were reviewed by a single genitourinary pathologist. Cases of pT3 UCRP were subclassified as pT3a (urothelial carcinomas extending only to the renal medulla) and pT3b (urothelial carcinomas extending into the renal cortex and/or peripelvic adipose tissue). Fine and Gray's model was used to predict recurrence-free survival (RFS) and cancer-specific survival (CSS). A total of 493 (51.7%) had UCRP and 461 (48.3%) had UCU. Within this group, 202 patients had pT3 UCRP and 146 had pT3 UCU. The pT3 subclassification of UCRP resulted in 79 cases of pT3a and 120 of pT3b. The difference in 5-year CSS among the pT3a UCRP, pT2 UCRP, and pT2 UCU subgroups was not statistically significant (pT3a UCRP vs pT2 UCRP, HR = 0.69, p = 0.56; pT3a UCRP vs pT2 UCU, HR = 0.66, p = 0.31) However, RFS and CSS were significantly higher in the pT3a UCRP group than in the pT3b group (pT3a vs pT3b, HR = 2.59, p = 0.0038 and pT3a vs pT3b, HR = 3.10, p = 0.001). The results suggest that our proposed pT3 subclassification better predicts the prognosis of UCRP patients than does the pT3 of the current AJCC/UICC classification.

Differential blood count as triage tool in evaluation of pelvic masses.

OBJECTIVE: Triaging patients with presumptive ovarian cancer to the appropriate specialist may improve survival. Therefore, there is increasing interest in complementary diagnostic markers to the standard serum CA125. In patients with pelvic masses, we examined the ability of epidemiologic variables and preoperative differential blood counts to improve detection of ovarian cancer over CA125 alone. METHODS: From pathology reports, patients were classified as having: epithelial ovarian cancer (n=743), including fallopian tube and primary peritoneal cancer, non-epithelial ovarian cancers (n=46), non-ovarian cancers (n=122), or benign disease (1,129). From women with epithelial ovarian cancer, we excluded those who received prior neoadjuvant chemotherapy (n=19). Women were also excluded if they did not have a serum CA125 or complete blood count measured within 180 days prior to surgery (n=1099) or did not have both tests within 90 days of each other (n=13). Categorizing patients by menopausal status, we calculated Pearson correlations between differential counts or ratios and CA125, and used t tests to identity univariate predictors of malignancy and stepwise logistic regression and likelihood ratio tests to create models best distinguishing epithelial ovarian cancer from benign disease. RESULTS: 337 women with epithelial ovarian cancer and 365 with benign disease were included in the analysis. Compared with cancers, women with benign disease had lower average: age, 52.5 versus 58.4 years (p<0.0001); serum CA125, 20 versus 239 U/mL (p<0.0001), neutrophil-to-lymphocyte ratio, 2.4 versus 3.5 (p<0.0001); and platelet-to-lymphocyte ratio, 158 versus 222 (p<0.0001); but greater average body mass index, 28.5 versus 26.8 kg/m2 (p=0.004), and lymphocyte-to-monocyte ratio, 5.6 versus 3.9 (p<0.0001). Correlations between counts and ratios and serum CA125 were seen in both epithelial ovarian cancer and benign disease groups and differed by menopausal status. In premenopausal women, a multivariate model including serum CA125, smoking, family history, lymphocytes, and monocytes performed similarly to the model with lymphocyte-to-monocyte ratio replacing counts. In postmenopausal women, a model including body mass index, parity, monocytes, and basophils performed similarly to the model replacing counts with platelet-to-lymphocyte ratio and lymphocyte-to-monocyte ratio. Models including epidemiologic variables and either counts or ratios were better at fitting data than models with serum CA125 and menopausal status alone. A single model applying to all women overstated performance for premenopausal women and understated performance for postmenopausal women. CONCLUSIONS: Epidemiologic variables and differential counts or ratios better distinguished between benign and malignant disease when compared with serum CA125 alone using separate models for pre- and postmenopausal women.

Adnexal masses in pregnancy: an updated review on diagnosis and treatment.

Adnexal masses are not common in pregnancy. They are often discovered incidentally during routine ultrasound examinations. In general, 24%-40% of the cases are benign tumors; up to 8% are malignant tumors. Adnexal masses are usually asymptomatic, but sometimes can be responsible for abdominal or pelvic pain. Transvaginal and transabdominal ultrasound is essential to define the morphology of pelvic masses and to distinguish between benign and malignant cases. Magnetic resonance imaging can be a complementary examination when ultrasound findings are equivocal and a useful additional examination to better define tissue planes and relations with other organs. Patient counseling can be challenging because there is no clear consensus on the management of adnexal masses during pregnancy. Treatment options consist of observational management (in case of asymptomatic women with reassuring instrumental findings) or surgery (via laparoscopy or laparotomy). Surgery can be offered as a primary tool when cancer is suspected or when acute complications such as ovarian torsion occur.

Reconstruction of Bony Defects after Tumor Resection with 3D-Printed Anatomically Conforming Pelvic Prostheses through a Novel Treatment Strategy.

There has been an increasing interest and enormous applications in three-dimensional (3D) printing technology and its prosthesis, driving many orthopaedic surgeons to solve the difficult problem of bony defects and explore new ways in surgery approach. However, the most urgent problem is without an effective prosthesis and standard treatment strategy. In order to resolve these problems, this study was performed to explore the use of a 3D-printed anatomically conforming pelvic prosthesis for bony defect reconstruction following tumor resection and to describe a detailed treatment flowchart and the selection of a surgical approach. Six patients aged 48-69 years who had undergone pelvic tumor resection underwent reconstruction using 3D-printed anatomically conforming pelvic prostheses according to individualized bony defects between March 2016 and June 2018. According to the Enneking and Dunham classification, two patients with region I+II tumor involvement underwent reconstruction using the pubic tubercle-anterior superior iliac spine approach and the lateral auxiliary approach and one patient with region II+III and three patients with region I+II+III tumor involvement underwent reconstruction using the pubic tubercle-posterior superior iliac spine approach. The diagnoses were chondrosarcoma and massive osteolysis. After a mean follow-up duration of 30.33 ± 9.89 months (range, 18-42), all patients were alive, without evidence of local recurrence or distant metastases. The average blood loss and blood transfusion volumes during surgery were 2500.00 ± 1461.51 ml (range, 1200-5000) and 2220.00 ± 1277.62 ml (range, 800-4080), respectively. During follow-up, the mean visual analogue scale (VAS) score decreased, and the mean Harris hip score increased. There were no signs of hip dislocation, prosthetic loosening, delayed wound healing, or periprosthetic infection. This preliminary study suggests the clinical effectiveness of 3D-printed anatomically conforming pelvic prostheses to reconstruct bony defects and provide anatomical support for pelvic organs. A new surgical approach that can be used to expose and facilitate the installation of 3D-printed prostheses and a new treatment strategy are presented. Further studies with a longer follow-up duration and larger sample size are needed to confirm these encouraging results.

Retrorectal cyst: proteus in the backyard-case series and literature review.

Retrorectal cysts are cystic lesions located in the retrorectal space and are a distinct subset of retrorectal tumours, which are often misdiagnosed due to their rarity and mimicry of symptoms caused by common diseases. We have described the presentation and management of four patients who were diagnosed with retrorectal cysts from a 10-year retrospective chart review at our institute, a tertiary care centre. In middle-aged women, the following should raise suspicion of retrorectal cyst: gastrointestinal or urinary obstructive features, mass or fullness palpable on the posterior wall on digital rectal examination, presacral dimple, perianal fistula and/or recurrent disease. Such features should prompt an MRI evaluation of the pelvis for definitive diagnosis.

Giant primitive neuroectodermal pelvic tumour: a case report and literature review.

Primitive neuroectodermal tumours (PNETs) are rare malignant small round cell tumours. Notably, despite widespread reports of PNET in multiple parts of the body, it is extremely rare in the pelvis. Here, a rare case of giant PNET of the pelvis, that was treated with surgical intervention comprising hemipelvectomy and amputation, is reported. A 42-year-old female patient presented with an enlarged mass on the left hip and severe pain in the left lower extremity for the previous 6 months. Preoperative imaging examinations indicated an irregular soft tissue-like signal shadow sized 19 × 15 × 12 cm at the left ilium and sacrum. After surgical intervention involving left hemipelvectomy and amputation, the tumour was diagnosed by pathology as PNET. During the courses of postoperative radiotherapy and chemotherapy, local recurrence and distant metastasis occurred, and the patient died 9 months following surgical treatment. To the best of the authors' knowledge, the current case is the largest pelvic PNET resection reported to date. Pelvic PNET is extremely malignant and has a high mortality rate regardless of surgical treatment, however, surgical resection of the lesion may relieve the symptoms, extend life, and improve quality of life to a certain extent.