Pelvic tumours are a rare cause of pleural effusion. We describe an approach to a case of Meigs syndrome with recurrent unilateral pleural effusion. A woman in her 60s\' presented with recurrent right-sided pleural effusion, leading to cough and shortness of breath. Thoracentesis yielded exudative pleural fluid with cytology negative for malignancy. Pleuroscopy revealed inflamed pleura, and pleural biopsy was consistent with inflammatory changes. The patient\'s cancer antigen 125 level was elevated at 256 U/mL. Given the high suspicion of malignancy, a computed tomography scan of the chest, abdomen, and pelvis was performed and revealed ascites and a large left ovarian and uterine mass. The patient underwent a total abdominal hysterectomy and bilateral salphingo oophorectomy after experiencing three additional episodes of pleural effusion. Histological examination revealed the left ovarian mass to be a cellular fibroma and the uterine masses to be leiomyomata. Following the operation, there was no recurrence of pleural effusion.

In 2002, heterozygous suppressor of fused variants (SUFU+/-) in the germline were described to have a tumor suppressor role in the development of pediatric medulloblastoma (MB). Other neoplasms associated with pathologic germline SUFU+/- variants have also been described among patients with basal cell nevus syndrome (BCNS; BCNS is also known as Gorlin syndrome, nevoid basal cell carcinoma [BCC] syndrome or Gorlin-Goltz syndrome; OMIM 109400), an autosomal-dominant cancer predisposition syndrome. The phenotype of patients with germline SUFU+/- variants is very poorly characterized due to a paucity of large studies with long-term follow-up. As such, there is a clinical need to better characterize the spectrum of neoplasms among patients with germline SUFU+/- variants so that clinicians can provide accurate counseling and optimize tumor surveillance strategies. The objective of this study is to perform a scoping review to map the evidence on the rate of medulloblastoma and to describe the spectrum of other neoplasms among patients with germline SUFU+/- variants. A review of all published literature in PubMed (MEDLINE), EMBASE, Cochrane, and Web of Science were searched from the beginning of each respective database until October 9, 2021. Studies of pediatric and adult patients with a confirmed germline SUFU+/- variant who were evaluated for the presence of any neoplasm (benign or malignant) were included. There were 176 patients (N = 30 studies) identified with a confirmed germline SUFU+/- variant who met inclusion criteria. Data were extracted from two cohort studies, two case-control studies, 18 case series, and eight case reports. The median age at diagnosis of a germline SUFU+/- variant was 4.5 years where 44.4% identified as female and 13.4% of variants were de novo. There were 34 different neoplasms (benign and malignant) documented among patients with confirmed germline SUFU+/- variants, and the most common were medulloblastoma (N = 59 patients), BCC (N = 21 patients), and meningioma (N = 19 patients). The median age at medulloblastoma diagnosis was 1.42 years (range 0.083-3; interquartile range 1.2). When data were available for these three most frequent neoplasms (N = 95 patients), 31 patients (32.6%) had neither MB, BCC nor meningioma; 51 patients (53.7%) had one of medulloblastoma or BCC or meningioma; eight patients (8.4%) had two of medulloblastoma or BCC or meningioma, and five patients (5.3%) had medulloblastoma and BCC and meningioma. This is the first study to synthesize the data on the frequency and spectrum of neoplasms specifically among patients with a confirmed germline SUFU+/- variant. This scoping review is a necessary step forward in optimizing evidence-based tumor surveillance strategies for medulloblastoma and estimating the risk of other neoplasms that could impact patient outcomes.

BACKGROUND: Nevoid basal cell carcinoma syndrome (NBCCS, Gorlin syndrome) is a rare autosomal dominantly inherited disorder that is characterized by multisystem disorder such as basal cell carcinomas, keratocystic odontogenic tumors and skeletal abnormalities. Bilateral and/or unilateral ovarian fibromas have been reported in individuals diagnosed with NBCCS.
METHODS: A 22-year-old female, presented with low back pain, and was found to have bilateral giant adnexal masses on pelvic ultrasonography, which had been suspected to be malignant ovarian tumors. Positron emission tomography/computed tomography showed multiple intracranial calcification and skeletal abnormalities. The left adnexa and right ovarian tumor were resected with laparotomy, and pathology revealed bilateral ovarian fibromas with marked calcification. We recommended the patient to receive genetic testing and dermatological examination. No skin lesion was detected. Germline testing identified pathogenic heterozygous mutation in PTCH1 (Patched1).
CONCLUSIONS: The possibility of NBCCS needs to be considered in patients with ovarian fibromas diagnosed in an early age. Skin lesions are not necessary for the diagnosis of NBCCS. Ovarian fibromas are managed with surgical excision with an attempt at preserving ovarian function. Follow-up regime and counseling on options for future fertility should be offered to patients.

Uterine leiomyoma should be considered when a female patient reports symptoms of abdominal pressure and abnormal vaginal bleeding. However, the symptoms of a uterine leiomyoma are vast and overlap with other possible diseases that are difficult to distinguish even with imaging studies. This is why it is important for physicians and healthcare providers to keep an open mind and have a broad differential diagnosis.  In this case study, we present a 61-year-old postmenopausal female patient who presented to the emergency department with complaints of pelvic and abdominal pain, as well as vomiting and diarrhea. She was admitted for observation. A complete blood count (CBC), comprehensive metabolic panel (CMP), and urinalysis revealed no abnormalities; a pelvic ultrasound and CT scan reported possible adnexal torsion. The patient remained stable and the pain had subsided when she was seen the next morning by her gynecologist (GYN) who discharged her to follow-up in the office. Subsequent examinations that aided in the diagnosis included, but were not limited to pelvic and transvaginal ultrasounds, an abdominal and pelvic CT, and a pelvic MRI. In this case, the MRI revealed an 11-cm mass that could represent a torsioned pedunculated necrotic fibroid originating from the uterus. Radiology recommended surgical removal. Upon removal and review of the pathology of the mass, it was revealed to be a torsioned, partially necrotic fibroma that had originated from the ovary and not from the uterus, as imaging had originally suggested.

Small ovarian fibromas (< 10cm) associated with elevated serum CA125 levels are rarely encountered, particularly in women of reproductive age. We report a rare case diagnosed in a 35-year-old patient after adnexectomy for a solid ovarian mass of approximately 5cm in maximum diameter, accompanied by elevated serum CA125 levels. In preoperative evaluation, no signs of inflammation from the genital tract were found, and no medical history of endometriosis, uterine leiomyomas, or non-gynecological cancer was reported. Intraoperative frozen section biopsy of surgical specimen obtained from the ovarian tumor had negative evaluation for malignancy. Histological examination of the surgical specimen confirmed the diagnosis of ovarian fibroma. The postoperative course was uneventful. Two months after surgery, the blood serum CA125 levels were within normal ranges. The patient is assessed at regular intervals in the gynecology outpatient clinic. In this paper, based on the data of the modern literature, a brief review of this rare nosological entity is made.

Ovarian fibroma is the most common benign pure stromal tumor. It has no specific clinical manifestation, most of which are pelvic or adnexal masses. 10-15% of cases with hydrothorax or ascites, after tumor resection, hydrothorax and ascites disappear, known as Meigs Syndrome. The elevated level of CA125 in a few patients was easily misdiagnosed as ovarian malignant tumor. A case of bilateral Ovarian fibroma associated with Meigs Syndrome is reported and the literature is reviewed in order to improve the understanding of the changes and avoid misdiagnosis.

UNASSIGNED: Nearly 90% of all the hormone-producing ovarian tumours are sex cord-stromal tumours (SCSTs). The Ovarian fibroma is a hormonally inactive variant of SCST. It is composed of spindle, oval, round cells producing collagen and accounts for approximately 4% of all ovarian neoplasms. Amongst the other SCSTs, Inhibin B is an important tumour marker. It is a heterodimeric glycoprotein hormone that is secreted primarily by the granulosa cells of the developing follicles. High levels of Inhibin-B can hamper follicular recruitment, leading to amenorrhea in a reproductive age woman.
UNASSIGNED: In this case report, we describe a rare case of a reproductive age female presenting with secondary amenorrhea, having an Ovarian Fibroma, producing massive amounts of Inhibin B.
UNASSIGNED: Although some pathological variants of ovarian fibromas like cyst-adeno-fibroma and ovarian fibro-thecoma are known to secrete inhibin B, benign /pure ovarian fibromas rarely do so.

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Basal cell nevus syndrome (BCNS) is a rare neurocutaneous syndrome characterized by tumorigeneses such as basal cell carcinomas, jaw cysts, ovarian fibromas, and cardiac fibromas. We present a 24-year-old female with calcified ovarian fibromas associated with BCNS. She had a surgical history of the maxillary cyst and was diagnosed with BCNS due to the cutaneous pits. Magnetic resonance imaging indicated an 8-cm mass and a 4-cm mass, which had been suspected to be a subserosal myoma and a fibroma, respectively. GnRH agonist was preoperatively administered; however, the size of the masses did not change. In laparoscopy, a tumor consisting of 8- and 5-cm masses in the right ovary was identified, and tumorectomy was performed. Because both tumors were extraordinarily rigid and could not be morcellated with scalpels or scissors, we removed them by the Luer Bone Rongeurs with minilaparotomy. The histopathological diagnosis was the ovarian fibromas with marked calcification.

Patients with Meigs\' syndrome and elevated serum CA-125 are not frequently reported. A 59-year-old woman and a 48-year-old woman sought help because of progressive shortness of breath caused by pleural effusion. The presence of a pelvic mass was noted in both the patients and was thought to be the cause of the effusion. Both patients had elevated serum CA-125, which raised the possibility of malignancy. After complete resection of the tumors, the pathologic reports confirmed a benign and a low-grade malignant ovarian neoplasia, respectively. We comment on the outcome and follow-up of these two cases and briefly review Meigs\' syndrome.