Multicenter Analysis of Presacral Neuroendocrine Neoplasms—Clinicopathological Characterization and Treatment Outcomes of a Rare Disease

Background and AimsNeuroendocrine neoplasms (NENs) of the presacral space are an extremely rare disease entity with largely unknown outcome and no established standard of care treatment. Therefore, we wanted to analyze clinical presentation, histopathological findings, treatment outcomes, and prognosis in a multicentric patient cohort.MethodsWe searched local databases of six German NEN centers for patients with presacral NEN. Retrospective descriptive analyses of age, sex, stage at diagnosis, symptoms, grade, immunohistochemical investigations, biomarkers, treatment, and treatment outcome were performed. Kaplan–Meier analysis was used to determine median overall survival.ResultsWe identified 17 patients (11 female, 6 male) with a median age of 50 years (range, 35–66) at diagnosis. Twelve cases presented initially with distant metastases including bone metastases in nine cases. On pathological review the majority of patients had well-differentiated G2 tumors. Immunohistochemical profile resembled rectal NENs. All but one patient had non-functioning tumors. Somatostatin receptor imaging was positive in 14 of 15 investigated cases. Eight patients were treated surgically including palliative resections; 14 patients received somatostatin analogs with limited efficacy. With 14 PRRTs completed, 79% showed clinical benefit, whereas only one patient with neuroendocrine carcinoma (NEC) responded to chemotherapy. Treatment with everolimus in three patients was not successful, whereas cabozantinib resulted in a disease stabilization in a heavily pretreated patient. During a median observation period of 44.5 months, 6 patients died. Median overall survival was not reached.ConclusionPresacral NEN are histopathologically similar to rectal NENs. Presacral NEN should be considered as possible primary in NEN of unknown primary. The majority of tumors is non-functioning and somatostatin receptor positive. PRRT demonstrated promising activity; tyrosine kinase inhibitors warrant further investigations. Further molecular characterization and prospective evaluation of this rare tumor entity are needed.

The Treatment Efficacy of Transparasacral Approach For Presacral Tumor: A Single Center Experience

Background: Presacral tumors, also known as retrorectal tumors, locate in the presacral space and are clinically rare and the approaches for the diagnosis and treatment of presacral tumors are still deficient. The four accepted surgical approaches for presacral tumors include transabdominal approach, sacrococcygeal approach, perineal approach, and combined approach. This study aims to evaluate the treatment efficacy of transparasacral approach for presacral tumors.Methods: 23 patients (7 males and 16 females) who are diagnosed with presacral tumors and receive surgery in transsacral approach at our department were chosen. The gender, age, body mass index (BMI), and clinical symptoms were recorded. The clinical data, postoperative complications, and short and long-term rehabilitation data were recorded and analyzed. Results: The surgery was performed smoothly and the patients recovered well without signs of intestinal fistula, abdominal and pelvic infection, lung infection, organ dysfunction, or organ failure. The incision edema occurred in only one patient. In terms of the histopathological types, presacral tumors can be classified into tailgut cyst (n=10), epidermoid cyst (n=5), teratoma (n=3), and lipoma (n=10). One patient diagnosed with tailgut cyst was accompanied by adenocarcinoma transition. Tumor recurrence didn’t occur in any patients. In addition, no patients complained about dysuria, sacrococcygeal sensory disturbance, or sacrococcygeal and perineal discomfort.Conclusions: Surgical resection with transparasacral approach is effective for the treatment of presacral tumors with the advantages of high safety, good treatment efficacy, few complications, good oncological prognosis, and fast recovery.

Expression of Estrogen and Progesterone Receptors in a Tailgut Cyst with Carcinoid Tumor

Introduction/Objective Tailgut cysts (TC) are congenital lesions that arise in the presacral space. They originate from the embryonic hindgut and usually present between the ages of 30-60, with female predominance. TCs are usually asymptomatic or can present with lower back, perianal or pelvic pain. Malignant transformation of a TC is a rare complication, with adenocarcinoma being the most common, followed by carcinoid tumor. About thirty cases of carcinoid tumors arising in a TC have been reported in the literature so far with a slight female predominance (1.5:1). Methods/Case Report We describe a 40-year-old Hispanic woman with a 9-year history of cyclic pelvic pain related to her menstrual cycles. She was diagnosed with adenomyosis and underwent hysterectomy that did not completely resolve her symptoms. Follow-up serial imaging showed a growing complex-cystic presacral mass, which was eventually excised. Grossly, there was a 3.6×3.1×2.5 cm multiloculated cystic mass filled with mucoid material. Microscopically, there were multiple cystic spaces lined by benign squamous and mucinous columnar epithelium and surrounded by smooth muscle cells. A 6-mm carcinoid tumor was found within a cyst wall and confirmed by positive synaptophysin and chromogranin staining with a Ki-67 proliferation index of <2%. Estrogen and progesterone receptor immunostains were positive in the epithelial cyst linings and the stromal cells but not in the carcinoid tumor cells. Results (if a Case Study enter NA) NA Conclusion We conclude that the diagnosis of TC should be considered in the differential of gynecologic patients with unresolved cyclic pelvic pain and that estrogen and progesterone could have an important role in the pathogenesis of TCs. Furthermore, estrogen receptors can be a therapeutic target in patients with this entity.

The Treatment Efficacy of Transparasacral Approach for Presacral Tumor: A Single Center Experience

Background Presacral tumors, also known as retrorectal tumors, locate in the presacral space and are clinically rare and the approaches for the diagnosis and treatment of presacral tumors are still deficient. The four accepted surgical approaches for presacral tumors include transabdominal approach, sacrococcygeal approach, perineal approach, and combined approach. This study aims to evaluate the treatment efficacy of transparasacral approach for presacral tumors. Methods 23 patients (7 males and 16 females) who are diagnosed with presacral tumors and receive surgery in transsacral approach at our department were chosen. The gender, age, body mass index (BMI), and clinical symptoms were recorded. The clinical data, postoperative complications, and short and long-term rehabilitation data were recorded and analyzed. Conclusions The surgery was performed smoothly and the patients recovered well without signs of intestinal fistula, abdominal and pelvic infection, lung infection, organ dysfunction, or organ failure. The incision edema occurred in only one patient. In terms of the histopathological types, presacral tumors can be classified into tailgut cyst (n = 10), epidermoid cyst (n = 5), teratoma (n = 3), and lipoma (n = 10). One patient diagnosed with tailgut cyst was accompanied by adenocarcinoma transition. Tumor recurrence didn’t occur in any patients. In addition, no patients complained about dysuria, sacrococcygeal sensory disturbance, or sacrococcygeal and perineal discomfort. Surgical resection with transparasacral approach is effective for the treatment of presacral tumors with the advantages of high safety, good treatment efficacy, few complications, good oncological prognosis, and fast recovery.

Supralevator Abscess Presenting With Pneumoarthrosis And Retroperitoneal Air, A Case Report

74-year-old male presented to the emergency department with complaint of fever, weakness, and fatigue and was admitted to the hospital for further workup. The patient was found to have gram negative bacteremia and Clostridioides difficile infection. Computed tomography imaging of the abdomen and pelvis was obtained and demonstrated air in the retroperitoneal/ presacral space tracking to the left hemipelvis and femoral joint. Concern for possible septic joint lead to an Orthopedic surgery evaluation and magnetic resonance imaging of the left hip and pelvis which revealed a complicated perirectal infection tracking to the left hemipelvis. The patient was taken to the operating room with colorectal surgery for further management and treatment.

Late presentation of extrauterine adenomyomas after laparoscopic morcellation at hysterectomy: a case report

Background An adenomyoma is a well circumscribed form of adenomyosis and can be located within the myometrium, in the endometrium as a polyp, or extrauterine with the last being the rarest presentation amongst the three. With the ongoing advancement in gynecological surgery, the use of electromechanical morcellators have made the removal of large and dense specimens possible with minimally invasive techniques. However, it has also caused an increase in complications which were previously rare. Whilst the tissue is being grinded within the abdominal cavity, residual tissue can spread and remain inside, allowing for implantation to occur and thereby giving rise to recurrence of uterine tissue as a new late postoperative complication. Case presentation A 45-year-old woman presented with worsening constipation and right iliac fossa pain. Her past surgical history consists of laparoscopic supra-cervical hysterectomy that was indicated due to uterine fibroids. Computerized tomography and magnetic resonance imaging were done, which showed an irregular lobulated heterogeneous mass seen in the presacral space to the right, located on the right lateral aspect of the recto-sigmoid, measuring 4.5 × 4.3 × 4.3 cm in size. A transvaginal ultrasound revealed a cyst in the left ovary. The patient had a treatment course over several months that included Dienogest (progestin) and Goserelin (GnRH analogue) with add-back therapy. In line with the declining response to medications, the patient was advised for a laparoscopic ovarian cystectomy. During the surgery, an additional lesion was found as a suspected fibroid and the left ovarian cyst was identified as pockets of peritoneal fluid which was sent for cytology. The surgical pathology report confirmed adenomyosis in both specimens, namely the right mass and the initially suspected fibroid. Conclusion In this case report, we showcase a rare occurrence of an extrauterine adenomyoma presenting two years post laparoscopic morcellation at hysterectomy. This poses questions regarding the benefits versus risks of power morcellation in laparoscopic hysterectomy.

The Treatment Efficacy of Transparasacral Approach For Presacral Tumor: A Single Center Experience

Background: Presacral tumors, also known as retrorectal tumors, locate in the presacral space and are clinically rare and the approaches for the diagnosis and treatment of presacral tumors are still deficient. The four accepted surgical approaches for presacral tumors include transabdominal approach, sacrococcygeal approach, perineal approach, and combined approach. This study aims to evaluate the treatment efficacy of transparasacral approach for presacral tumors.Methods: 23 patients (7 males and 16 females) who are diagnosed with presacral tumors and receive surgery in transsacral approach at our department were chosen. The gender, age, body mass index (BMI), and clinical symptoms were recorded. The clinical data, postoperative complications, and short and long-term rehabilitation data were recorded and analyzed. Conclusions: The surgery was performed smoothly and the patients recovered well without signs of intestinal fistula, abdominal and pelvic infection, lung infection, organ dysfunction, or organ failure. The incision edema occurred in only one patient. In terms of the histopathological types, presacral tumors can be classified into tailgut cyst (n=10), epidermoid cyst (n=5), teratoma (n=3), and lipoma (n=10). One patient diagnosed with tailgut cyst was accompanied by adenocarcinoma transition. Tumor recurrence didn’t occur in any patients. In addition, no patients complained about dysuria, sacrococcygeal sensory disturbance, or sacrococcygeal and perineal discomfort. Surgical resection with transparasacral approach is effective for the treatment of presacral tumors with the advantages of high safety, good treatment efficacy, few complications, good oncological prognosis, and fast recovery.

Surgical Resection of a Presacral Solitary Fibrous Tumor with Extension to Iliac Vessels using Karakousis’s Abdominoinguinal Approach: Report of a Rare Case

Background: The solitary fibrous tumor (SFT) is a rare tumor of mesenchymal origin, with a reported incidence of 2.8 cases per 100.000 tumors and with distinctive histopathological and immunohistochemical characteristics. It was initially described as a pleural lesion and subsequently, it was found in different organs and tissues. The abdominoinguinal incision described by Karakousis allows a radical reliable approach for abdominal-pelvic tumors in lower quadrants. We present the case of a patient with a presacral STF extending to iliac vessels, which required the Karakouis’s approach for its surgical resection.Case Presentation: Forty-seven-year-old man with pain in lumbosacral region radiating to left leg since 5 months. MRI and CT show evidence of a 10cm-retroperitoneal mass (presacral space) extending to left iliac vessels. The early diagnosis corresponded to a sarcomatous retroperitoneal tumor. It was decided to carry out the abdominal scan through the Karakousis’s approach for surgical resection with permanent neurovascular control. The immunohistochemistry and histopathological study revealed a CD-34 positive spindle-cell neoplasia, vimentin, Bcl-2, and β-catenin, compatible with a SFT. It was categorized as low risk for developing metastasis and death from disease, according to the new criteria for malignancy. At present, the patient is asymptomatic and disease-free at 17 months after surgery.Conclusion: Presacral SFT is a rare entity, with a scant incidence reported regarding this location and long-term treatment. Surgical resection is needed as the immediate treatment.

Presacral Tumor: Insights From a Decade’s Experience of This Rare and Diverse Disease

BackgroundPresacral tumors are a group of rare and heterogeneous tumors that arise from the potential presacral space between the rectum and sacrum. The low occurrence and diverse origins make the diagnosis and treatment of these tumors a challenge. The aim of the study was to retrospectively review patient demographics and to identify advantages and disadvantages in the diagnosis and treatment of these tumors.MethodsRetrospectively collected and reviewed data from patients who received treatment of presacral tumors at the First Affiliated Hospital of China Medical University between August 2009 and June 2019.ResultsThe data from forty-four patients (33 females) with a median age of 50 years who were diagnosed with a presacral/retrorectal tumor were analyzed. The majority of tumors were congenital (61.4%) and benign tumors are more common (59.1%). The median age of patients with benign tumor was significantly higher than that of malignant tumor. The most common symptoms were sacrococcygeal/perianal pain (56.8%) and mass (36.4%), and 8 out of 9 patients having lower limb symptoms diagnosed with malignant tumor. The tumor detection rate of digital rectal examination was 75% and more than 90% of all patients underwent one or more radiology imaging exams for tumor diagnosis. Every patient had a biopsy result. The most common type of tumor was presacral cyst (40.9%) with overall tumor median size of 5.6 cm. Thirty-one (70.5%) patients underwent surgery, most often via the posterior route (83.9%). Posterior route surgery had significantly shorter operation time and tumors operated via posterior route were significantly smaller. The survival rate after surgery was 100%. The median course of disease was 6 months and median follow-up was 25 months.ConclusionsPresacral tumors have low occurrence and are more frequently observed in females in their 30s and 50s indicating a possible link between tumor occurrence and hormonal changes. Patients with lower limb symptoms were more likely to have a malignant presacral tumor. Posterior route was the most commonly utilized surgical approach. Supplementary iodine tincture treatment of cysts ruptured in operation could potentially be helpful in reducing the chance of recurrence.