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Ophthalmic Plastic and Reconstructive...Two healthy 12-year-old monozygotic twin sisters presented with strikingly similar, painless orbital masses along their frontozygomatic suture line that had been slowly...
Two healthy 12-year-old monozygotic twin sisters presented with strikingly similar, painless orbital masses along their frontozygomatic suture line that had been slowly enlarging since birth. The masses were clinically consistent with orbital dermoid cysts and the patients underwent excision of their lesions, with the diagnosis confirmed by histological analysis. There are prior case reports of both nasal and ovarian dermoid cysts in twins, however, no prior case of orbital dermoid cysts in twins have been described. These dermoid cysts are generally thought to be a sporadic disorder of embryogenesis, yet the authors' case suggests genetics may play a role in the underlying etiology of dermoid cysts.
Topics: Child; Female; Humans; Dermoid Cyst; Nose; Orbital Neoplasms; Tomography, X-Ray Computed; Twins, Monozygotic
PubMed: 37133396
DOI: 10.1097/IOP.0000000000002401 -
Open Veterinary Journal Sep 2023Teratomas are rare types of germ cell neoplasms composed of various differentiated or undifferentiated tissues. (Review)
Review
BACKGROUND
Teratomas are rare types of germ cell neoplasms composed of various differentiated or undifferentiated tissues.
CASE DESCRIPTION
A 25-week-old female control FVB /n mouse in a 4-week toxicity study presented abdominal distension and poor body condition. It was euthanized, and the necropsy examination revealed a large mass connected to the tip of the right uterine horn, occupying the entire abdominal cavity. Microscopically, this mass showed areas of epidermal differentiation, with laminated keratin and sebaceous glands, differentiation into respiratory and digestive epithelium, cartilage, bone, and extensive areas of differentiation into the nervous tissue, being classified as an ovarian teratoma.
CONCLUSION
As far as authors know, the occurrence of ovarian teratomas in the FVB/n mouse strain has never been previously described.
Topics: Mice; Female; Animals; Ovarian Neoplasms; Teratoma; Rodent Diseases
PubMed: 37842105
DOI: 10.5455/OVJ.2023.v13.i9.19 -
EClinicalMedicine Mar 2024Ovarian immature teratoma (IT) is a rare neoplasm comprising ∼3% of ovarian cancers, occurring primarily in young females. Management presents several challenges,... (Review)
Review
Ovarian immature teratoma (IT) is a rare neoplasm comprising ∼3% of ovarian cancers, occurring primarily in young females. Management presents several challenges, including those with elevated serum alpha-fetoprotein, potential confusion regarding pathology interpretation, and paucity of data to support decision-making. MaGIC (https://magicconsortium.com/) is an interdisciplinary international consortium of GCT experts from multiple subspecialties, with members receiving frequent queries regarding IT patient management. With evidence from published literature where available, we summarise consensus management of such patients. Given lack of published data, controversy in certain areas remains. The most obvious variance in practice is between paediatric and adult teams, despite very similar outcomes. Paediatric teams typically employ a surgery-only approach, whereas in adult practice, all patients, except those with stage IA, grade 1 (low-grade) tumours, still generally receive adjuvant chemotherapy. Given the rarity of ovarian IT and lack of published data, discussion with GCT experts and/or national advisory panels is recommended.
PubMed: 38544795
DOI: 10.1016/j.eclinm.2024.102453 -
World Journal of Radiology Jan 2024Anti-N-methyl-D-aspartate receptor-associated encephalitis (NMDARE) is a rare immune-mediated neuroinflammatory condition characterized by the rapid onset of... (Review)
Review
Anti-N-methyl-D-aspartate receptor-associated encephalitis (NMDARE) is a rare immune-mediated neuroinflammatory condition characterized by the rapid onset of neuropsychiatric symptoms and autonomic dysfunction. The mechanism of pathogenesis remains incompletely understood, but is thought to be related to antibodies targeting the GluN1 subunit of the NMDA receptor with resultant downstream dysregulation of dopaminergic pathways. Young adults are most frequently affected; the median age at diagnosis is 21 years. There is a strong female predilection with a female sex predominance of 4:1. NMDARE often develops as a paraneoplastic process and is most commonly associated with ovarian teratoma. However, NMDARE has also been described in patients with small cell lung cancer, clear cell renal carcinoma, and other benign and malignant neoplasms. Diagnosis is based on correlation of the clinical presentation, electroencephalography, laboratory studies, and imaging. Computed tomography, positron emission tomography, and magnetic resonance imaging are essential to identify an underlying tumor, exclude clinicopathologic mimics, and predict the likelihood of long-term functional impairment. Nuclear imaging may be of value for prognostication and to assess the response to therapy. Treatment may involve high-dose corticosteroids, intravenous immunoglobulin, and plasma exchange. Herein, we review the hallmark clinicopathologic features and imaging findings of this rare but potentially devastating condition and summarize diagnostic criteria, treatment regimens, and proposed pathogenetic mechanisms.
PubMed: 38312349
DOI: 10.4329/wjr.v16.i1.1 -
Medicine May 2024Trophoblastic neoplasms are often associated with pregnancy, and nongestational trophoblastic neoplasms are extremely rare. Nongestational ovarian choriocarcinoma (NGCO)... (Review)
Review
INTRODUCTION
Trophoblastic neoplasms are often associated with pregnancy, and nongestational trophoblastic neoplasms are extremely rare. Nongestational ovarian choriocarcinoma (NGCO) is a highly aggressive germ cell-derived tumor frequently presenting with early hematogenous metastasis.
PATIENT CONCERNS
Herein, we report a case of a 28-year-old unmarried woman with regular menstruation who experienced vaginal bleeding 1 week after her last menstrual cycle. Doppler ultrasound revealed bilateral adnexal masses and elevated serum human chorionic gonadotropin (hCG) levels. The patient was initially misdiagnosed as presenting an ectopic pregnancy.
DIAGNOSIS
The final pathology confirmed an International Federation of Gynecology and Obstetrics stage IA NGCO with bilateral mature teratoma of the ovary. This is an extraordinary instance of ovarian choriocarcinoma which emerged without any prior gestation, and the patient's lack of a history of pregnancy made the diagnosis ignored.
INTERVENTIONS
After initial surgery and 1 cycle of bleomycin, etoposide, and cisplatin (BEP) chemotherapy, a laparoscopic fertility-preserving comprehensive staging surgery was performed. Two cycles of chemotherapy with BEP were administered as supplemental therapy postsurgery, and leuprorelin was administered to protect ovarian function.
OUTCOMES
Menstruation resumed 4 months after chemotherapy completion, and tumor indicators were within the normal range. No signs of recurrence were observed at the 36-month follow-up.
CONCLUSION
NGCO should be considered if a female patient exhibits irregular vaginal bleeding and masses in the adnexal area. The present case and our literature review also highlighted that fertility-sparing surgery and multidrug chemotherapy are effective methods for treating NGCO.
Topics: Humans; Female; Adult; Ovarian Neoplasms; Teratoma; Choriocarcinoma, Non-gestational; Antineoplastic Combined Chemotherapy Protocols; Etoposide; Pregnancy; Bleomycin
PubMed: 38701311
DOI: 10.1097/MD.0000000000036996 -
Taiwanese Journal of Obstetrics &... Sep 2023To report a rare case of anti-N-Methyl-d-aspartate receptor encephalitis (anti-NMDARE) presented by mental and behavioral changes and seizures accompanied with...
OBJECTIVE
To report a rare case of anti-N-Methyl-d-aspartate receptor encephalitis (anti-NMDARE) presented by mental and behavioral changes and seizures accompanied with respiratory failure.
CASE REPORT
A 37-year-old multiparous woman was initially presented with abnormal mental behavior and the diagnosis of schizophrenia was made, but the disease progressed rapidly to general convulsion and acute respiratory failure. Although active treatment, including steroids, intravenous immunoglobulins (IVIGs) and plasma exchange was applied, no significant improvement was obtained. Transvaginal ultrasound and pelvic magnetic resonance image (MRI) were arranged and the results showed a suspicious cystic lesion (3 × 2.3 cm) at the right ovary. Laparoscopic unilateral salpingo-oophorectomy was performed and final pathology reported a matured cystic teratoma, suggesting that this patient had anti-NMDARE secondary to ovarian mature teratoma. After surgery, the clinical condition was dramatically improved and she recovered completely without sequelae.
CONCLUSION
Although it is well-known about the relation between anti-NMDARE and ovarian mature teratoma, this small tumor may result in the missing diagnosis. Remind us to consider the possibility of any small ovarian cystic lesion-related anti-NMDARE in women with autoimmune encephalitis.
Topics: Female; Humans; Adult; Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Teratoma; Ovarian Neoplasms; Respiratory Distress Syndrome
PubMed: 37679011
DOI: 10.1016/j.tjog.2023.07.024 -
Cureus Feb 2024We present a rare case of a 45-year-old woman with pseudo-Meigs' syndrome and eosinophilic pleural effusion (EPE). She experienced cough, sputum, and dyspnea with a...
We present a rare case of a 45-year-old woman with pseudo-Meigs' syndrome and eosinophilic pleural effusion (EPE). She experienced cough, sputum, and dyspnea with a large right pleural effusion. Laboratory tests showed eosinophilia in the blood and pleural fluid. An ovarian tumor and ascites were also detected. After left salpingo-oophorectomy, the tumor was diagnosed as a mature cystic teratoma of the left ovary. The right-sided pleural effusion gradually resolved. Pseudo-Meigs' syndrome is characterized by benign ovarian tumor, ascites, and pleural effusion. Typically, it is associated with exudate pleural effusion characterized by a predominance of mononuclear cells. The occurrence of eosinophilic pleural effusion in our patient may be exceptionally rare.
PubMed: 38523926
DOI: 10.7759/cureus.54686 -
Journal of Minimal Access Surgery Jan 2024Majority of adnexal masses are incidental findings during pregnancy and resolve spontaneously. They may complicate pregnancy due to haemorrhage, cyst rupture and ovarian...
BACKGROUND
Majority of adnexal masses are incidental findings during pregnancy and resolve spontaneously. They may complicate pregnancy due to haemorrhage, cyst rupture and ovarian torsion. Laparoscopy is the preferred surgical intervention owing to shorter operative time, quicker recovery and lower post-operative pain. However, safety and feasibility of laparoscopic surgery in the management of ovarian cysts in pregnant women is challenging especially at advanced gestational ages and in urgent situations.
METHODS
In this retrospective study, a total of 48 cases of urgent laparoscopic surgeries were evaluated in the first and second trimesters of pregnancy between March 2018 and March 2021 in Al-Zahra Hospital, Tabriz, Iran.
RESULTS
The operations were ended in all patients successfully. The mean operation time was 45 min. There were no foetal maternal complications. All pregnancies had been terminated after 37 weeks of gestation. Oophorectomy had been performed in six patients and six other patients needed blood transfusion. Ovarian torsion was the most common reason for emergency surgery and mature teratoma was the most commonly reported pathology. Tocolytic therapy was required in six patients, and all of the patients were prescribed 50 mg of injectable progesterone twice daily for 10 days after surgery. The mean intra-abdominal pressure was equal to 15 mmHg and the mean end-expiratory carbon dioxide (CO 2 ) pressure was 36 mmHg. Intravenous paracetamol was used to relieve post-operative pain. The mean hospitalisation time was 1.63 days.
CONCLUSION
Most of the ovarian cysts can be managed laparoscopically with ensured safety and lower morbidity even in emergency situations at advanced gestational ages.
PubMed: 37706405
DOI: 10.4103/jmas.jmas_192_22 -
Molecular and Cellular Endocrinology Mar 2024Endocrine cells responsible for hormone secretion are found in virtually every organ system. The diverse neoplasms arising from endocrine cells in the female...
Endocrine cells responsible for hormone secretion are found in virtually every organ system. The diverse neoplasms arising from endocrine cells in the female reproductive tract are not well recognized as a distinct component of endocrine oncology. Here, we integrate cellular origins with native anatomical residence to help classify neoplasms of this system. The neoplasms include steroidogenic tumors that arise usually in ovarian stroma, neuroendocrine neoplasms that can arise from normal neuroendocrine cells throughout the female reproductive tract or in ovarian germ cell tumors, and thyroid follicular cell proliferations that are exclusively a component of an ovarian teratoma and may be malignant. The neuroendocrine neoplasms run the full spectrum from indolent neuroendocrine tumors to aggressive poorly differentiated neuroendocrine carcinomas. While many of these lesions are identified as incidental findings in surgically resected tissues, others present with inappropriate hormone excess. An important consideration is the distinction of primary disease from metastatic malignancy. Genetic disorders including those caused by germline mutations of the FOXL2, GNAS, DICER1, STK11 and MEN1 genes can present with primary endocrine neoplasms of the female reproductive tract.
Topics: Humans; Female; Ovarian Neoplasms; Neuroendocrine Tumors; Teratoma; Germ-Line Mutation; Hormones; Ribonuclease III; DEAD-box RNA Helicases
PubMed: 38135144
DOI: 10.1016/j.mce.2023.112123 -
Medicine Aug 2023Carcinoid tumors, derived from the cells of the disseminated neuroendocrine system, are rare, slow-growing neuroendocrine neoplasms that display a relatively indolent...
RATIONALE
Carcinoid tumors, derived from the cells of the disseminated neuroendocrine system, are rare, slow-growing neuroendocrine neoplasms that display a relatively indolent disease course. The majority of carcinoids are found within the gastrointestinal tract and bronchopulmonary system. Primary ovarian carcinoids are rare and account for merely 1% of all carcinoid tumors. We describe our experience of a rare case of primary ovarian carcinoid, presenting as chronic constipation, with no other carcinoid symptoms such as flushing, diarrhea, and wheezing.
PATIENT CONCERNS
A 51-year-old postmenopausal woman with chronic constipation visited the clinic for routine check-up of her preexisting uterine fibroids. She had undergone hemorrhoidectomy 3 years ago. Physical examination revealed a soft abdomen without direct or rebound tenderness. Transvaginal ultrasonography revealed two subserosal fibroids, which had increased in size compared with previous ultrasonographic findings. A 3 cm hyperechoic mass was also detected in the right ovary. Her blood and urine tests were unremarkable, with no ascites in the pelvic cavity. She had a normal CA-125 level of 5.5 units/mL.
DIAGNOSIS, INTERVENTIONS, AND OUTCOMES
The patient underwent a robot-assisted hysterectomy and right salpingo-oophorectomy because of enlarging fibroids and the right ovarian mass. Subsequently, based on the pathological and immunohistochemical findings, she was diagnosed with a primary ovarian carcinoid. The mass consisted of the insular and trabecular types of tumor cells. It was positive for pan-cytokeratin and synaptophysin, and the Ki-67 proliferation index was less than 1%. A follow-up positron emission tomography-computed tomography revealed no distant metastasis. Six months postoperatively, the patient was doing well without any signs of recurrence.
LESSONS
Primary ovarian carcinoids without teratoma components are rare. It is crucial to make an accurate diagnosis based on the immunohistochemical staining results. Diagnosis in the early stages of the disease are associated with a favorable prognosis, but regular follow-up is mandatory.
Topics: Humans; Female; Middle Aged; Ovarian Neoplasms; Salpingo-oophorectomy; Carcinoid Tumor; Constipation
PubMed: 37543794
DOI: 10.1097/MD.0000000000034391