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Journal of the Turkish German... Jun 2024Gliomatosis peritonei (GP) is a rare entity characterized by multiple mature glial tissue implants in association with ovarian teratomas in the peritoneum and omentum....
OBJECTIVE
Gliomatosis peritonei (GP) is a rare entity characterized by multiple mature glial tissue implants in association with ovarian teratomas in the peritoneum and omentum. To date, only 100 cases have been published. Not much is known about the origin, clinicopathological profile or prognosis of GP. SOX2 and OCT4 are recently recognized markers of embryonic stem cell differentiation. Here, the role of SOX2 and OCT4 in the pathogenesis of 11 cases of GP are reported and clinicopathological factors are described.
MATERIAL AND METHODS
This was a retrospective study of six years duration (2017-2022). All the cases of GP were retrieved from archives, the diagnosis was confirmed and clinicopathological factors were noted. Immunohistochemical (IHC) investigation for glial fibrillary acid protein (GFAP) and S100 was noted wherever available. IHC for SOX2 and OCT4 was performed using an avidin-biotin technique.
RESULTS
There were 11 cases of GP identified. The median age was 29 years and 1/11 cases had nodal gliomatosis as well. There were eight cases of immature teratoma and three cases of mature cystic teratoma. SOX2 was positive in all foci of GP, while OCT4 was negative. These foci were also positive for GFAP and S100.
CONCLUSION
A possibility of GP should be considered as a differential, clinically and radiologically, in cases of omental nodularity. Adequate sampling at the time of surgery is essential to rule out metastasis or growing teratoma syndrome. SOX2, a stem cell marker inducing neural differentiation, may play a crucial role in the development of GP in association with other transcription factors.
PubMed: 38867687
DOI: 10.4274/jtgga.galenos.2024.2023-9-4 -
Case Reports in Women's Health Jun 2024Mature cystic teratomas (MCTs) arise from totipotent cells. While the ovaries are the most common sites, extragonadal teratomas are very rare. This case report describes...
Mature cystic teratomas (MCTs) arise from totipotent cells. While the ovaries are the most common sites, extragonadal teratomas are very rare. This case report describes the clinical details of a 20-year-old woman who was found to have a large pelvi-abdominal mass on clinical and imaging studies and elevated levels of CA-125. Because of the uncertainties of the origin of the mass, a multidisciplinary team suggested exploratory laparotomy with a mesenteric cyst as a differential diagnosis. Laparotomy showed both ovaries to be normal and revealed a large omental MCT, which was excised. Histopathology confirmed the diagnosis.
PubMed: 38855718
DOI: 10.1016/j.crwh.2024.e00619 -
Oncology Letters Jul 2024The onset of two synchronous primary malignancies of the female genital tract is uncommon; therefore, the simultaneous occurrence of cervical small cell neuroendocrine...
The onset of two synchronous primary malignancies of the female genital tract is uncommon; therefore, the simultaneous occurrence of cervical small cell neuroendocrine carcinoma and ovarian immature teratoma is rare. The present study describes the case of a woman with cervical small cell neuroendocrine carcinoma complicated by ovarian immature teratoma. The clinical manifestations, and the histopathological and immunophenotypic features of the patient are recorded. Furthermore, all PubMed-indexed cases of synchronous primary malignancies in both the cervix and ovary have been briefly summarized.
PubMed: 38807671
DOI: 10.3892/ol.2024.14446 -
Journal of Family & Reproductive Health Dec 2023Malignant transformation in dermoid cysts is rare, and Squamous Cell Carcinoma (SCC) is the most common form. This event often occurs in large tumors and middle-aged...
OBJECTIVE
Malignant transformation in dermoid cysts is rare, and Squamous Cell Carcinoma (SCC) is the most common form. This event often occurs in large tumors and middle-aged women.
CASE REPORT
In this study, two cases are presented. They were menopause, and abdominal pain and adnexal mass was a common manifestation in both. Case 1 with adenocarcinoma arising in mature cystic teratoma had abnormal tumor markers and was diagnosed with a frozen section during surgery, but case 2 with SCC transformation had normal tumor markers, and the frozen section was not helpful in the first surgery. Both underwent complete staging surgery, and due to stage IC1 in case 1, she received chemotherapy, and in case 2, no adjuvant treatment was needed because of stage IA.
CONCLUSION
Considering the rarity of malignant transformation in the dermoid cyst, the best surgical approach and adjuvant therapy indications need further research.
PubMed: 38807623
DOI: 10.18502/jfrh.v17i4.14599 -
The American Journal of Case Reports May 2024BACKGROUND Malignant peritoneal mesothelioma is a rare disease with a poor prognosis that often presents with vague symptoms and inconclusive laboratory test results....
BACKGROUND Malignant peritoneal mesothelioma is a rare disease with a poor prognosis that often presents with vague symptoms and inconclusive laboratory test results. Causes include industrial pollutants, primarily asbestos, and certain genetic mutations, such as BAP1. Due to the nonspecific symptoms, it is often incidentally diagnosed during or after other surgical procedures. CASE REPORT A 35-year-old healthy woman underwent an uncomplicated laparoscopic left salpingo-oophorectomy for a symptomatic large ovarian mature cystic teratoma. She subsequently presented with late-onset postoperative fever, leukocytosis, and multiple intra-abdominal masses. Following an exploratory laparotomy, extensive infectious disease evaluation, and multiple biopsies requiring interdisciplinary collaboration, malignant peritoneal mesothelioma was diagnosed by positive histologic staining of an omental biopsy for D2-40 and CK5/6. This first specimen was positive for BAP1, with the second, a liver biopsy, testing negative for BAP1. The tumor cell testing was also notable for mutations in NF2, MLL2, and ARID1A, and the hereditary cancer genetic testing was overall unremarkable. Her disease progressed rapidly, and she died 6 months after her initial procedure. CONCLUSIONS This case of rapidly developing malignant peritoneal mesothelioma following surgical management of an ovarian mature teratoma highlights the complexity in diagnosing a rare disease that presents with nonspecific symptoms in an otherwise young and healthy woman. The rapid disease course was likely accelerated by expansive intraperitoneal spread and multiple somatic oncogenic mutations in BAP1, NF2, MLL2, and ARID1A. Gynecologists should keep a broad differential for postoperative complications, as occult malignancies can present with symptoms that mimic postoperative complications.
Topics: Humans; Female; Adult; Peritoneal Neoplasms; Postoperative Complications; Ovarian Neoplasms; Mesothelioma, Malignant; Fatal Outcome; Diagnosis, Differential; Disease Progression; Teratoma; Salpingo-oophorectomy; Mesothelioma
PubMed: 38803090
DOI: 10.12659/AJCR.942948 -
Heliyon May 2024This study aimed to assess the surgical outcomes and identify the conversion risk factors of Transvaginal Natural Orifice Transluminal Endoscopic Surgery (vNOTES) in...
OBJECTIVE
This study aimed to assess the surgical outcomes and identify the conversion risk factors of Transvaginal Natural Orifice Transluminal Endoscopic Surgery (vNOTES) in treating ovarian cyst.
METHODS
This was a retrospective study of 505 patients who underwent vNO TES for treating ovarian cyst from March 2019 to February 2022 wherein the patients were classified into "converted" or "nonconverted" groups. T-tests, χ tests, and logistic regression were used for statistical analyses.
RESULTS
There were 16 (3.17 %) surgical conversions and 12 (2.38 %) other surgical complications in our study cohort. Teratomas accounted for 56.8 % of complications in nonconverted cases and 18.8 % in converted cases. Adenocystomas were found in 12.3 % of nonconverted cases and 18.8 % of converted cases. Other types included paraovarian cysts (3.3 % and 0 %), fibroma, granulosa cell tumor, Brenner tumor (1.2 % and 0 %), corpus luteum cysts, follicular cysts (7.6 % and 6.3 %), old abscess (0.2 % and 0 %), and simple cysts (17.6 % and 12.5 %) in the nonconverted and converted groups, respectively. The converted group included more cases of endometriotic cysts (43.8 % vs 12.3 %, p = 0.023), bilateral cysts (37.5 % vs 8.2 %, p < 0.001), severe pelvic adhesion (68.8 % vs 3.3 %, p < 0.001), deep endometriosis (12.5 % vs 0.4 %, p < 0.001), and at least two cysts (37.5 % vs 8.81 %; p < 0.001). Severe pelvic adhesion (adjusted odds ratio [OR], 86.96; range, 18.33-431.77; p < 0.001), bilateral cysts (adjusted OR, 4.75; range, 1.05-21.57, p = 0.043) and endometriotic cysts (adjusted OR, 7.69; range, 3.11-17.08; p < 0.001) were also predictors of surgical conversion.
CONCLUSION
vNOTES demonstrates low complication and conversion rates in treating ovarian cyst compared with TU-LESS. Surgical conversion is associated with severe pelvic adhesions, bilateral cysts, and endometriotic cysts.
PubMed: 38774314
DOI: 10.1016/j.heliyon.2024.e31014 -
Frontiers in Immunology 2024Anti-NMDA receptor encephalitis is an autoimmune disorder caused by autoantibodies (abs) against the conformational epitope on GluN1 subunits. GluN1-abs have been...
INTRODUCTION
Anti-NMDA receptor encephalitis is an autoimmune disorder caused by autoantibodies (abs) against the conformational epitope on GluN1 subunits. GluN1-abs have been determined with cell-based assay (CBA) co-expressing GluN1/GluN2 subunits. However, commercial fixed CBA expressing only GluN1 subunit has increasingly been used in clinical practice. The ab titers can be determined with serial dilutions, but its clinical significance remains unclear. We aimed to develop an H-intensity scale (HIS) score to estimate GluN1-ab titers in cerebrospinal fluid (CSF) with one-time immunostaining using both commercial CBA and immunohistochemistry and report its usefulness. "H" is the initial of a patient with high CSF GluN1-ab titers (1:2,048).
METHODS
We first determined the reliability of CBA in 370 patients with suspected autoimmune encephalitis by comparing the results between commercial CBA and established assay in Dalmau's Lab. Then, we made positive control panels using the patient H's CSF diluted in a fourfold serial dilution method (1:2, 1:8, 1:32, 1:128, 1:512, and 1:2,048). Based on the panels, we scored the intensity of ab reactivity of 79 GluN1-ab-positive patients' CSF (diluted at 1:2) on a scale from 0 to 6 (with ≥1 considered positive). To assess inter-assay reliability, we performed immunostaining twice in 21 patients' CSF. We investigated an association between the score of CSF obtained at diagnosis and the clinical/paraclinical features.
RESULTS
The sensitivity and specificity of CBA were 93.7% (95% CI: 86.0-97.3) and 98.6% (95% CI: 96.5-99.5), respectively. Linear regression analysis showed a good agreement between the scores of the first and second assays. Patients with a typical spectrum, need for mechanical ventilation support, autonomic symptoms/central hypoventilation, dyskinesias, speech dysfunction, decreased level of consciousness, preceding headache, ovarian teratoma, and CSF leukocyte count >20 cells/µL had a higher median HIS score than those without, but HIS score was not associated with sex, age at onset, or seizure. HIS score at diagnosis had a significant effect on 1-year functional status.
DISCUSSION
The severity of disease and four of the six core symptoms were associated with higher GluN1-ab titers in CSF at diagnosis, which may play a role in poor 1-year functional status. An incomplete phenotype can be attributed to low CSF GluN1-ab titers.
Topics: Humans; Female; Autoantibodies; Middle Aged; Adult; Male; Receptors, N-Methyl-D-Aspartate; Aged; Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Young Adult; Adolescent; Child; Immunohistochemistry; Child, Preschool; Nerve Tissue Proteins; Reproducibility of Results; Biomarkers; Aged, 80 and over
PubMed: 38745654
DOI: 10.3389/fimmu.2024.1350837 -
Cureus Apr 2024Struma ovarii is a monodermal teratoma characterized by the presence of >50% thyroid tissue. It is mostly benign; therefore, preoperative diagnosis is important. It...
Struma ovarii is a monodermal teratoma characterized by the presence of >50% thyroid tissue. It is mostly benign; therefore, preoperative diagnosis is important. It usually manifests as a multilocular cystic mass but rarely as a predominantly solid mass. On magnetic resonance imaging (MRI), solid-appearing struma ovarii showed early signal intensity enhancement on dynamic gadolinium-enhanced T1-weighted images, which histopathologically indicates the presence of thyroid tissue with abundant blood vessels. The Ovarian-Adnexal Reporting and Data System (O-RADS) MRI score is a validated classification worldwide for characterizing adnexal lesions. Based on the morphology, signal intensity, and enhancement of any solid tissue on the MRI, the scoring system can be used to classify adnexal lesions into five categories from score one (no adnexal mass) to score five (high risk of malignancy). An adnexal solid mass with a higher signal intensity than that of the myometrium 30-40 seconds after gadolinium (Gd) injection on non-dynamic contrast-enhanced (non-DCE) MRI was assigned a score of 5 (high risk of malignancy). We present a case of solid-appearing struma ovarii with a higher signal intensity than that of the myometrium 30 seconds after Gd injection on non-DCE MRI, and it was classified as score five preoperatively. Therefore, a total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed despite the presence of a benign ovarian mass. When an adnexal mass with a higher signal intensity than that of the myometrium 30-40 seconds after Gd injection on non-DCE MRI is encountered, struma ovarii should be included in the differential diagnosis, despite the O-RADS MRI score of five and management of the situation should be discussed.
PubMed: 38741801
DOI: 10.7759/cureus.58176 -
Cureus Apr 2024This study aims to investigate the co-existence of ovarian teratomas with other benign or malignant gynecological tumors in women who underwent gynecological surgery.
INTRODUCTION
This study aims to investigate the co-existence of ovarian teratomas with other benign or malignant gynecological tumors in women who underwent gynecological surgery.
METHODS
We retrospectively reviewed all women who underwent gynecological surgery over a 15-year period. Pre-operative, surgical, and histological records were obtained from women who presented with gynecological pathology, aiming to discover a possible link between ovarian teratomas and other gynecological tumors.
RESULTS
Of the total patient sample, 288 (8.2%) had a mature teratoma, and 9 (0.3%) had an immature teratoma. The mean age was 38.0±13.3 years and 30.9±11.1 years, respectively. Women with mature teratoma showed a positive correlation with struma ovarii (SO, p=0.001). Moreover, we reported a positive linear relationship between struma ovarri and thecoma. Of the 288 women with a mature teratoma, 1 (0.3%) had co-existent endometrioid ovarian cancer, and 1 (0.3%) had borderline cancer. There were 14 women (4.9%) with a co-existent serous cystadenoma, 7 (2.4%) with a mucin cystadenoma, 1 (0.3%) with a thecoma, 4 (1.4%) with struma ovarii, 3 (1.0%) had Brenner cyst, 3 (1.0%) had ovarian fibroma, 2 had endometriosis (0.7%), and 8 (2.8%) had endometriomas. Of a total of nine women with immature teratomas, one (11.1%) had a serous cystadenoma.
CONCLUSIONS
Ovarian teratomas may co-exist with other gynecological diseases. Our study reports various cases of the co-existence of several gynecological tumors with teratomas.
PubMed: 38737998
DOI: 10.7759/cureus.58068 -
Case Reports in Women's Health Jun 2024NMDA-R encephalitis is an autoimmune encephalitis that is known to be associated with ovarian teratomas. Eighty to 100 % of patients initially present with...
NMDA-R encephalitis is an autoimmune encephalitis that is known to be associated with ovarian teratomas. Eighty to 100 % of patients initially present with neuropsychiatric symptoms. Early recognition and intervention are critical to management and prognosis. This case demonstrates non-specific presenting symptoms of NMDA-R encephalitis. A 32-year-old woman presented to the emergency room with headache, nausea, vomiting, and photophobia. She was discharged with probable aseptic meningitis. Eight days later, she represented with delusional thought content, perseverative speech, and bizarre behavior. Cerebrospinal fluid studies showed elevated protein and mild pleocytosis. A computed tomography scan with contrast showed a 35-mm complex cystic lesion in the right adnexa, which was resected. Confirmatory pathology showed a mature cystic teratoma. Paraneoplastic panel later resulted positive for NMDA-R encephalitis. The patient was treated with methylprednisolone, IVIG, plasmapheresis, and rituximab. The clinical course was complicated by a hypersensitivity reaction to rituximab, non-convulsive status epilepticus requiring intubation, dysphagia requiring a PEG placement, a rectal ulcer causing acute blood loss anemia requiring multiple blood transfusions, bilateral hearing loss, and a left lung pneumothorax. The patient's mood, cognition, and motor function were favorably improving 19 months after diagnosis. This case illustrates presenting signs of NMDA-R encephalitis in a young woman as headache and altered mental status followed by psychosis and epilepsy. Treatment should involve a multidisciplinary team and be individualized and escalated in patients with worsening clinical status refractory to first-line therapy. Further research is warranted to understand the optimal treatment strategy for this disease.
PubMed: 38737718
DOI: 10.1016/j.crwh.2024.e00612