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Gynecologic Oncology Reports Jun 2023While Non-Hodgkin Lymphoma (NHL) often involves the ovaries at time of autopsy, it is rarely present at the time of diagnosis. Here we present a case of a 20-year-old...
While Non-Hodgkin Lymphoma (NHL) often involves the ovaries at time of autopsy, it is rarely present at the time of diagnosis. Here we present a case of a 20-year-old who presented with a large adnexal mass and elevated B-HCG, CA-125, and LDH. The patient underwent exploratory laparotomy, and frozen section of the left ovarian mass was suspected to be a dysgerminoma. Final pathologic diagnosis was Ann Arbor stage IVE Diffuse Large B-Cell Lymphoma, germinal center subtype. Patient is currently undergoing chemotherapy and has received the 3 of a planned 6 cycles of R-CHOP.
PubMed: 37293350
DOI: 10.1016/j.gore.2023.101212 -
Pediatrics International : Official... Jan 2022
Topics: Dysgerminoma; Female; Humans; Neoplasms, Germ Cell and Embryonal; Neoplasms, Second Primary; Ovarian Neoplasms; Teratoma
PubMed: 35851512
DOI: 10.1111/ped.15251 -
Modern Pathology : An Official Journal... Jul 2022Mast cell sarcoma (MCS) is an exceedingly rare form of mastocytosis characterized by invasive malignant mast cell growth and metastatic potential. Diagnosis of MCS is... (Review)
Review
Mast cell sarcoma (MCS) is an exceedingly rare form of mastocytosis characterized by invasive malignant mast cell growth and metastatic potential. Diagnosis of MCS is very challenging due to its marked morphologic variations and significant immunophenotypic overlap with other neoplasms. In this study, we undertook an extensive study of 10 cases of MCS from our series, with review of additional 24 cases from the literature, to better clarify the clinicopathologic and molecular features of MCS. From the analyses of our 10 cases, MCS equally involved males and females with a median age of 54.5 years (range 1-63). The bone was the most common site of involvement, as noted in 9/10 of cases. Two patients had prior germ cell tumors (mediastinal germ cell tumor and ovarian dysgerminoma), and concurrent systemic mastocytosis was noted in one of nine patients. Serum tryptase levels were elevated in 6/7 of patients, and 3/9 of patients had mast cell activation symptoms. Morphologically, the tumor cells were typically large and pleomorphic with frequent reactive eosinophils. By immunohistochemical staining, MCS consistently expressed CD43 (8/8), CD117 (10/10), and mast cell tryptase (10/10), as well as CD13 (3/3) and CD33 (10/10), with variable positivity of CD2 (1/9), CD25 (4/9), CD30 (5/8), and CD68 (5/9). Notably, KIT D816V was not detected in nine cases in our study, although two cases had other mutations of KIT gene. Seven out of eight patients received chemotherapy with or without radiotherapy. However, the response was poor, and four out of eight patients died within a median follow-up interval of five months. Taken together, there are no standardized therapeutic regimens available for MCS at this time, and the prognosis is dismal. Therefore, it is critical to further investigate and characterize this rare entity, with the hope of improving diagnostic accuracy and providing more effective, targeted therapies.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Infant; Male; Mast Cells; Mast-Cell Sarcoma; Mastocytosis; Mastocytosis, Systemic; Middle Aged; Proto-Oncogene Proteins c-kit; Young Adult
PubMed: 35105959
DOI: 10.1038/s41379-022-01014-w -
Medicine Oct 2020Dysgerminoma is an extraordinarily rare neoplasm arising from the malignant germ cells of the ovary. Early antenatal diagnosis and proper management of the neoplasm to... (Review)
Review
RATIONALE
Dysgerminoma is an extraordinarily rare neoplasm arising from the malignant germ cells of the ovary. Early antenatal diagnosis and proper management of the neoplasm to improve maternal-neonatal results are the considerable challenges facing the gyne-oncologist. We summarize the clinical features and discuss treatment strategies of the ovary dysgerminoma (OD). Besides, we also review the literature on OD in PubMed, Web of Science Core Collection, Library of Congress, and LISTA from 1939 to 2019 to evaluate its clinical characteristics, feto-maternal compromise, management, and fertility outcome.
PATIENT CONCERNS
A 25-year-old pregnant woman reported lower abdominal pain and vomiting.
DIAGNOSIS
The patient was diagnosed as right OD.
INTERVENTIONS
She received a cesarean section due to severe abdominal pain, delivered a healthy girl at 38 C 4 weeks of gestation, and accepted fertility-preserving surgery. However, the patient refused chemotherapy postoperatively.
OUTCOMES
The patient was followed up 42 days, 3 months, and 6 months after surgery, and no tumor recurrence was observed.
LESSONS
OD has non-specificity characteristics, including age, symptoms, image date, and tumor marks. However, these abnormal indicators may provide some evidence for accurate antenatal diagnosis. The management strategies should be considered comprehensively on an individual basis, and fertility-preserving surgery should be carried out in the second trimester if further pregnancy is desired. Adjuvant chemotherapy needs to be applied to the treatment of OD patients with The International Federation of Gynecology and Obstetrics (FIGO) stages II, III, and IV and timely chemotherapy is suggested if there are several weeks before the expected date of delivery. The overall prognosis of OD patients is excellent.
Topics: Adult; Cesarean Section; Dysgerminoma; Female; Humans; Ovarian Neoplasms; Pregnancy; Pregnancy Outcome
PubMed: 33031254
DOI: 10.1097/MD.0000000000021214 -
Maedica Dec 2021Patients with Swyer syndrome, "XY gonadal dysgenesis", have fibrosed gonads with a significant risk of developing germ cell tumours. During radiological assessment, a...
Patients with Swyer syndrome, "XY gonadal dysgenesis", have fibrosed gonads with a significant risk of developing germ cell tumours. During radiological assessment, a 17-year-old female with Swyer syndrome showed mildly enlarged gonads that were removed laparoscopically and proved pathologically to be bilateral gonadoblastomas. In addition, the right sided lesion showed overgrowth by dysgerminoma. The patient was further treated with combination chemotherapy and she was in complete remission for three years.
PubMed: 35261680
DOI: 10.26574/maedica.2020.16.4.734 -
Asian Journal of Surgery Dec 2023
Topics: Female; Adolescent; Humans; Dysgerminoma; Ovarian Neoplasms
PubMed: 37657981
DOI: 10.1016/j.asjsur.2023.08.150 -
Medicine Apr 2021Although dysgerminomas are relatively uncommon among all ovarian neoplasms, representing for only about 2%, they account for 32.8 percent of malignant ovarian germ cell...
RATIONALE
Although dysgerminomas are relatively uncommon among all ovarian neoplasms, representing for only about 2%, they account for 32.8 percent of malignant ovarian germ cell tumors. Their association with pregnancy is extremely rare; due to the low frequency of occurrence, there are few recommendations regarding pregnancy management; therefore, it is important to discuss and summarize the treatment strategy.
PATIENT CONCERNS
We present the case of a 25 years patient, gestation 1, para 1, who was hospitalized in the clinic at 38/39 weeks of gestation at the beginning of labor. Following the ultrasound examination, a hypoechogenic lesion on the uterine fundus was found, suggestive of subterranean fibroid. After caesarean section, right adnexectomy was performed; the histopathological examination revealed, unexpectedly, the diagnosis of dysgerminoma.
DIAGNOSES
Dysgerminoma as associated with pregnancy.
INTERVENTIONS
Birth by Caesarean section and right adnexectomy. No other medical complications occurred.
OUTCOMES
The histopathological and immunohistochemical examinations were consistent with the pure dysgerminoma. Oncology was staged AI, with the monitoring of markers and abdominal and pelvic magnetic resonance imaging at 3, 6, 9, and 12 months.
LESSONS
Dysgerminoma is the most common ovarian malignancy associated with pregnancy with a good fetal maternal outcome. If these tumors are discovered accidentally during caesarean section, tumor markers and magnetic resonance imaging scanning should be done postoperatively to plan optimal treatment.
Topics: Adult; Aftercare; Biomarkers, Tumor; Cesarean Section; Dysgerminoma; Female; Humans; Magnetic Resonance Imaging; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Pregnancy; Pregnancy Complications, Neoplastic; Treatment Outcome; Ultrasonography
PubMed: 33832117
DOI: 10.1097/MD.0000000000025364 -
Pediatric Blood & Cancer Jun 2023
Topics: Female; Humans; Child; Meigs Syndrome; Dysgerminoma; Ovarian Neoplasms; Ascites; Diagnosis, Differential
PubMed: 36721998
DOI: 10.1002/pbc.30224 -
A Comprehensive Review of Current Trends in the Diagnosis and Treatment of Ovarian Germ Cell Tumors.Cureus Jan 2024Ovarian germ cell tumors constitute a rare and intricate spectrum of neoplasms characterized by diverse histological subtypes. This comprehensive review elucidates the... (Review)
Review
Ovarian germ cell tumors constitute a rare and intricate spectrum of neoplasms characterized by diverse histological subtypes. This comprehensive review elucidates the classification, diagnosis, treatment, prognosis, and unique challenges associated with these tumors. The classification is rooted in histological attributes, with principal subtypes encompassing dysgerminoma, immature teratoma, yolk sac tumor (endodermal sinus tumor), choriocarcinoma, and mixed germ cell tumors. Each subtype bears distinct characteristics and clinical implications, necessitating precise diagnosis and tailored therapeutic strategies. Diagnosis hinges upon recognizing the broad clinical presentation, employing imaging techniques (such as ultrasound and MRI), evaluating tumor markers (alpha-fetoprotein and beta-human chorionic gonadotropin), and conducting histopathological examinations where necessary. Staging, primarily utilizing the International Federation of Gynecology and Obstetrics (FIGO) system, is pivotal in determining the extent of disease, guiding treatment choices, and facilitating prognostic assessment. Treatment modalities encompass surgery, chemotherapy (including standard regimens and emerging therapies), radiation therapy, targeted therapies, and immunotherapy. Prognosis is influenced by histological subtype, tumor stage, patient age, surgical success, response to chemotherapy, and tumor markers, while predictive biomarkers are continually emerging. Despite advances in treatment, ovarian germ cell tumors pose distinct challenges, including late diagnosis, treatment-related side effects, and the enigma of chemoresistance. An integral aspect of comprehensive care is supportive strategies to manage symptoms and offer psychological and emotional support. This review accentuates the vital role of early diagnosis and multidisciplinary care in optimizing outcomes. Future research directions and evolving clinical practices are explored in these intricate and distinctive malignancies, highlighting the dynamic landscape of ovarian germ cell tumors.
PubMed: 38380211
DOI: 10.7759/cureus.52650 -
Radiologic Clinics of North America Jul 2023Ovarian malignant germ cell tumors are a diverse set of masses originating from the primitive gonadal germ cells, often in young females. They have useful imaging and... (Review)
Review
Ovarian malignant germ cell tumors are a diverse set of masses originating from the primitive gonadal germ cells, often in young females. They have useful imaging and clinical features, including serum tumor marker elevation, that may aid the radiologist at the time of diagnosis, and also during follow-up. Accurate and timely diagnosis is essential, as standard-of-care therapies lead to a high rate of cancer remission.
Topics: Female; Humans; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Biomarkers, Tumor
PubMed: 37169425
DOI: 10.1016/j.rcl.2023.02.004