Borderline Serous Papillary Tumor Arising in a Paraovarian Cyst: A Case Report and an Extensive Review of the Literature

Background: Paraovarian cysts are benign cysts that develop near the ovaries and fallopian tubes in the pelvic region. They can cause mass effects requiring excision. In rare cases, tumors may develop inside these cysts. These tumors may be benign, malignant, or borderline. Case Report: A 26-year-old lady presented for excision of a paraovarian cyst. Pre-operative imaging showed the presence of few undulating folds at the periphery of the cyst. The patient underwent laparoscopic pelvic cystectomy without intra-operative drainage. Pathological examination of the specimen revealed a borderline serous papillary tumor protruding from the cyst wall. Conclusion: The occurrence of a borderline tumor, also known as a tumor of low malignant potential, in a pre-existing paraovarian cyst is very rare and has only been reported few times in the literature. A thorough review of these cases showed that the most common imaging finding that raises suspicion for a borderline tumor within a paraovarian cyst is the presence of small intracystic projections within the unilocular adnexal cyst. However, since evaluating the presence of an intracystic tumor is not always possible, performing a fertility-preserving laparoscopic cystectomy without cyst content spillage, is recommended. If properly excised, the prognosis of this tumor is good, and recurrence is rare.

P14.89 Papillary tumor of the pineal region: three cases from a single institution

BACKGROUND Papillary tumors of the pineal region are highly unusual lesions of unknown incidence. These are neuroepithelial tumors with papillary and solid areas. There is no standardized treatment because of the difficulty to make randomized prospective trials due to its rarity. We report three patients with papillary tumor of the pineal region. MATERIAL AND METHODS Retrospective review of the clinical records of patients with papillary tumor of the pineal region at a tertiary neurological center in Buenos Aires, Argentina was done between 2/2012-04/2021. Clinical characteristics, imaging, pathology, treatment and follow-up data were obtained. RESULTS Two patients were male and one female. All three patients were adults, two in the fourth decade of life and one in the seventh. Clinical presentation included headache along with either visual symptoms, gait disturbance, or both. Imaging workup included brain and spinal magnetic resonance imaging, finding expanding lesions in the pineal region, hyperintense in FLAIR/T2 sequences, with gadolinium enhancement in T1 with contrast sequence and variable degrees of hydrocephalus. Spinal lesions were not found in these patients. Cerebrospinal fluid cytology was negative in all cases. Tumor biopsy was the initial approach in all cases. Two patients underwent an initial third ventriculostomy procedure but all were submitted to a supracerebellar infratentorial approach for major resection. Posterior treatment included chemoradiotherapy with temozolomide. One patient had a distant recurrence after seven years with and remained with stable disease for two more years, and the other two patients are under control with no recurrence after 2 and 2.5 years respectively. CONCLUSION Papillary tumors in the pineal regions are aggressively treated as recurrence is very frequent regarding previous reports (> 70%). Total resection is the only predictive factor associated to better outcome, however, more information is needed in order to achieve treatment consensus.

Papillary tumor of the pineal region: Is stereotactic radiosurgery efficient for this rare entity?

Background: Papillary tumors of the pineal region are rare neuroepithelial lesions that were described for the 1st time in the WHO 2007 classification. Management of such lesions remains controversial. Case Description: We describe the case of a 26-year-old female who presented with intracranial hypertension syndrome secondary to a 1.9 cm3 lesion of the pineal region causing hydrocephalus. The patient benefited from an endoscopic third ventriculocisternostomy and a biopsy of her lesion in favor of a papillary tumor of the pineal region. After discussion of the surgical risks, the patient refused the surgical option and a stereotactic radiosurgery (SRS) was performed. She improved both clinically (allowing her to regain autonomy) and radiologically (reduction of 60% of tumor volume) at 1 year follow-up. Conclusion: Because of the rarity of the lesion, literature is yet not able to find consensus concerning management of such lesion, but SRS has proven efficiency for these Grades II or III lesions with high recurrence rates. Therefore, it should be considered as a primary therapeutic option allowing good outcome with low risks for the patient.

A rare case of ciliated muconodular papillary tumor accompanied with adenocarcinoma in situ

Background Ciliated muconodular papillary tumor (CMPT) is an incredibly rare pulmonary tumor. Currently, little is known about CMPT, and it has not yet been classified by the World Health Organization. The clinical manifestation of CMPT is nonspecific and the diagnosis is only based on pathology. CMPT has been documented in limited reports as a benign tumor, thus the treatment is typically with surgical excision if a solid tumor is identifiable. The prognosis of CMPT is very positive, as no recurrence has been reported in the limited literature available. However, CMPT accompanied with adenocarcinoma in situ has not been reported previously in the literature. Case presentation In this report, we presented a case of a 53-year-old male smoker with CMPT associated with adenocarcinoma in situ. This diagnosis was confirmed by pathological examination, including immunohistostaining. No solid resectable lesion was identified on CT scan; therefore, no surgery was performed. The patient’s adenocarcinoma in situ was disseminated in both lungs, thus chemotherapeutic treatment with cisplatin and pemetrexed was given. The patient will be continually followed up closely on a wait-and-watch basis. Conclusions In summary, our report reveals a unique case of CMPT in conjunction with adenocarcinoma in situ, potentially revealing an association between CMPT and malignancy which has not been previously reported. More similar case studies will be beneficial to determine the authentic relationship between CMPT and adenocarcinoma in situ.

Retractorless interforniceal approach for microsurgical resection of a papillary tumor of the pineal region: operative video and technical nuances

In this illustrative video, the authors demonstrate microsurgical resection of a papillary tumor of the pineal region using a retractorless interforniceal approach via the anterior interhemispheric transcallosal route. The tumor presented to the posterior third ventricle occluding the cerebral aqueduct, resulting in obstructive hydrocephalus. The retractorless interforniceal approach is performed in the lateral position with BICOL collagen spacers to keep the corridor open. Gross-total resection was achieved, and the patient was neurologically intact without needing a permanent shunt. The operative nuances and pearls of technique for safe microdissection and gentle handling of the retractorless interforniceal approach are demonstrated. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2139.

Resection of a pineal region papillary tumor using robotic exoscope: improved visualization and ergonomics for deep seeded tumor

Surgery for pineal region tumors is technically challenging due to their deep location and close proximity to critical deep venous structures, midbrain, and thalamus. A high-definition video exoscope was recently proposed as an alternative to the operating microscope. The authors illustrate a case of the midline supracerebellar infratentorial approach to resect a pineal region tumor using the Modus V exoscope and demonstrate the improved visualization of critical structures in this deep location. Additionally, the marked improvement in surgeon comfort suggests that this system may have significant advantages over traditional microscope-based surgery for tumors of the pineal region. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2127.