A Surgical Approach Decision-Making for Benign Presacral Tumors in Adult Women: A Case Series

Background: The incidence of benign presacral tumor is relatively low in adults and is much higher in women than in men. However, by far, there is no standard surgical approach for women. The study hope to propose a reasonable surgical approach decision strategy exclusively for adult women, which is feasible for removing tumors in different sizes and different locations. Methods: 4 adult female patients with benign presacral tumor were admitted to our department for surgical treatment over these 5 years. Their tumors were removed by transvaginal, laparoscopic, and combined laparoscopic-vaginal surgery respectively. Results: The operations were all carried out successfully. Patients who have undergone repeated examinations in the follow-up after surgery have no tumor recurrence. Conclusion: A reasonable surgical approach decision-making is beneficial to the surgical procedure and post-operation recovery. Transvaginal approach is an uncommon but important approach for benign presacral tumors in adult female.

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.

Neuroendocrine Tumor Arising in a Tailgut Cyst: A Rare Presacral Tumor

A tailgut cyst (retrorectal cystic hamartoma) is an uncommon lesion that develops in the presacral (retrorectal) space. Malignant change in a tailgut cyst is extremely rare and presents as a soft tissue (presacral) or bone (sacral) neoplasm. We report a case of tailgut cyst in which a neuroendocrine tumor developed in a 25-year-old female. Computed tomography and magnetic resonance imaging scans revealed a sacrococcygeal malformation with absent left S4 and S5 and a partly cystic lesion within the right presacral space. Histologically, the lesion contained cystic and solid elements. The cysts were lined by columnar and stratified squamous epithelial cells with underlying patchy smooth muscle. The solid element was a partly necrotic neuroendocrine tumor composed mainly of ribbons of tumor cells, which showed mitotic activity and expressed cytokeratin, chromogranin, and synaptophysin. Histologically, tailgut cysts are lined by epithelium and contain scattered smooth muscle bundles in the cyst wall. Although rare, the possibility of tailgut cyst with neuroendocrine tumor should be included in the differential diagnosis of an enlarging presacral tumor.

Rapid onset obesity and ondine’s curse: a deadly syndrome

ROHHADNET syndrome is characterized by rapid-onset-obesity, hypoventilation, hypothalamic dysregulation, autonomic dysfunction and neural tumors. A 2.4-year-old girl presented with inability to be aroused from sleep. She was obese, obtunded, hypoventilating with severe hypercarbia. Non-invasive ventilation (NIV) was started following which her sensorium, hypercarbia normalized. History revealed hyperphagia, rapidly increasing weight after 1½ year of age with normal height centiles. Hormonal profile revealed hyperprolactinemia, central hypothyroidism, suboptimal growth hormone response and normal cortisol. She had presacral tumor, pain insensitivity, fluctuating blood pressure, constipation and strabismus. ROHHADNET syndrome was clinically diagnosed based on constellation of these features. She was discharged on nocturnal NIV. Over 8 months, her hypoventilation progressed requiring tracheostomy. She also underwent excision of the presacral tumor which proved to be ganglioneuroma. Whole exome sequencing was negative for CCHS and ROHHAD genes, which could be due to somatic mosaicism; variation in the genomic region not covered by the test; or large insertions, deletions, complex rearrangements. Arriving at the diagnosis is difficult due to its overlap with other hyperphagic obesity syndromes and lack of confirmatory genetic testing.

An Unusual Fat-Containing Presacral Tumor in an Elderly Patient

The authors present a case of a presacral myelolipoma diagnosed in an 84-year-old male patient with longstanding pelvic pain and past medical history of bladder cancer. Pelvic computed tomography (CT) revealed a well-encapsulated and lobulated presacral mass, with mixed fat and soft-tissue attenuation. Magnetic resonance (MR) imaging provided further confirmation of macroscopic intralesional fat and excluded either adjacent bone invasion or bladder cancer recurrence. A presacral myelolipoma was suspected based on imaging findings, with liposarcoma and teratoma having also been considered for the differential diagnosis. The histological confirmation of the tumor was only attained postoperatively. This case report alerts to the possible presacral location of myelolipomas, which should be considered for every fat-containing lesion detected in this region. The main clinical, imaging, and differential diagnoses of this entity are reviewed in this paper.