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Journal of Education & Teaching in... Jan 2022A 5-month-old female presented to the emergency department with worsening abdominal distention and postprandial emesis. Point-of-care ultrasound (POCUS) demonstrated...
UNLABELLED
A 5-month-old female presented to the emergency department with worsening abdominal distention and postprandial emesis. Point-of-care ultrasound (POCUS) demonstrated extensive abdominal free fluid with a large, heterogeneous mass in the suprapubic region. This was confirmed on computed tomography (CT) of the abdomen and pelvis. The patient was urgently taken to the operating room for exploratory laparotomy where a ruptured tumor was discovered. The patient was eventually diagnosed with ovarian juvenile granulosa cell tumor. POCUS can be vital in guiding the recognition of rare pediatric conditions in the emergency department and should be considered in patients where there is concern for an acute abdomen.
TOPICS
Abdominal pain, ascites, ovarian juvenile granulosa cell tumor, point-of-care ultrasound.
PubMed: 37483405
DOI: 10.21980/J8035H -
BMC Pediatrics Aug 2023Hepatocellular adenomas (HCAs) are rare benign tumors of the liver that occur predominantly in women taking oral contraceptives. In children, HCAs comprise < 5% of... (Review)
Review
BACKGROUND
Hepatocellular adenomas (HCAs) are rare benign tumors of the liver that occur predominantly in women taking oral contraceptives. In children, HCAs comprise < 5% of hepatic tumors. We report a case of HCAs in a 7-year-old girl with estrogen and glucose imbalance.
CASE PRESENTATION
A 7-year-old girl was presented to our hospital with bilateral breast enlargement for 2 months, polydipsia, polyuria, polyphagia, hyperglycemia, and significant weight gain. Computed tomography (CT) showed a 7.2 cm×6.9 cm×5.3 cm round-shaped mass in the left inner lobe of the liver, ovarian ultrasound showed multiple follicles in the ovaries bilaterally, and cranial magnetic resonance imaging (MRI) showed an enlarged superior pituitary. Hematological and biochemical results were as follows: fasting glucose was 19.7 mmol/L, estradiol was 122.9 pmol/L, follicle-stimulating hormone 10.81 IU/L, luteinizing hormone 10.99 IU/L, insulin-like growth factor 1,513 ng/mL, glutamine aminotransferase 86 U/L, and alkaline phosphatase 362 U/L. Thyroid functions, methemoglobin, fetal protein, carcinoembryonic antigen, and chorionic gonadotropin were normal. The patient had a complete surgical resection of the liver tumor, and the postoperative histopathological diagnosis was HCAs. After the surgery, insulin was injected and the glucose levels were stable. During the 36-month follow-up period, neither tumor recurrence nor significant abnormalities were detected using color Doppler ultrasound of the liver. The child's precocious puberty is currently under control.
CONCLUSIONS
HCAs are particularly rare in children with liver tumors, and risk factors for the development of HCAs in children include sex hormone imbalance, obesity, Fanconi anemia (FA), glycogen storage diseases (GSDs) type I, III, and IV, galactosemia, immunodeficiency, congenital portosystemic shunts (CPSS), cardiac hepatopathy status-post Fontan procedure, Hurler syndrome, familial adenomatous polyposis, germline HNF1A mutations, and maturity-onset diabetes of the young type 3. Most HCAs are detected during a physical examination without clinical symptoms, and some patients may present with symptoms such as abdominal pain, abdominal distension, and abdominal masse. Serum liver function tests can show increased alkaline phosphatase (ALP) and γ- glutamyl transferase (GT), whereas α-Fetoprofein (AFP) levels are normal. The definitive diagnosis relies mainly on histopathological examination. Because HCAs can rupture and bleed and become malignant. Early surgical treatment is recommended after detection.
Topics: Child; Humans; Female; Adenoma, Liver Cell; Alkaline Phosphatase; Neoplasm Recurrence, Local; Liver Neoplasms
PubMed: 37620840
DOI: 10.1186/s12887-023-04209-5 -
Archives of Gynecology and Obstetrics May 2022Pregnancy complicated with juvenile granulosa cell tumor (JGCT) is very rare; thus, the experience on clinical diagnosis and management is limited. (Review)
Review
BACKGROUND
Pregnancy complicated with juvenile granulosa cell tumor (JGCT) is very rare; thus, the experience on clinical diagnosis and management is limited.
CASES
Two patients presented with abdominal pain, two were incidentally discovered, one by ultrasonography, and one during a caesarian section. One case received an emergency caesarian section because of tumor rupture at 38th week's gestation, the rest were treated at full term and no abnormalities were detected in the newborns. Three cases received further staging surgery, two of which received postoperative adjuvant chemotherapy. No patient had recurrent disease after a follow-up period spanning from 13 to 57 months.
CONCLUSION
In the absence of emergency, surgery can be delayed without affecting the fetus. More research is needed to determine the value of chemotherapy in FIGO stage I patients.
Topics: Chemotherapy, Adjuvant; Female; Granulosa Cell Tumor; Humans; Infant, Newborn; Neoplasm Staging; Ovarian Neoplasms; Pregnancy; Ultrasonography
PubMed: 34694430
DOI: 10.1007/s00404-021-06283-5 -
Comparative Biochemistry and... Apr 2021Melatonin, the neurohormone mainly synthesized in and secreted from the pineal gland of vertebrates following a circadian rhythm, is an important factor regulating... (Review)
Review
Melatonin, the neurohormone mainly synthesized in and secreted from the pineal gland of vertebrates following a circadian rhythm, is an important factor regulating various physiological processes, including reproduction. Recent data indicate that melatonin is also synthesized in the ovary and that it acts directly at the level of the ovary to modulate ovarian physiology. In some teleosts, melatonin is reported to affect ovarian steroidogenesis. The direct action of melatonin on the ovary could be a possible factor promoting oocyte maturation in teleosts. A role for melatonin in follicle rupture during ovulation in the teleost medaka has recently emerged. In addition, melatonin is suggested to affect oocyte maturation by its antioxidant activity. However, the molecular mechanisms underlying these direct effects of melatonin are largely unknown.
Topics: Animals; Antioxidants; Circadian Rhythm; Female; Fishes; Melatonin; Neurotransmitter Agents; Oocytes; Oogenesis; Ovarian Follicle; Ovary; Pineal Gland; Reactive Oxygen Species; Steroids
PubMed: 33482340
DOI: 10.1016/j.cbpa.2021.110907 -
The British Journal of Radiology Jan 2021Corpus luteum rupture presenting as acute abdomen is an underdiagnosed condition. Though a self-limiting entity, its differentiation from other causes is essential to... (Review)
Review
Corpus luteum rupture presenting as acute abdomen is an underdiagnosed condition. Though a self-limiting entity, its differentiation from other causes is essential to prevent unnecessary surgical procedures. The radiologist should be aware of the possibility of a ruptured haemorrhagic ovarian cyst in a female of reproductive age group presenting with pelvic pain and a large amount of haemorrhagic ascites. Imaging characteristically reveals a thick-walled cystic structure in the adnexa with internal echoes, focal discontinuity or irregularity of its wall with haemoperitoneum. While sonography is usually indicative of corpus luteum rupture, cross-sectional imaging (CT/MRI) can be used to confirm the diagnosis.
Topics: Abdomen, Acute; Corpus Luteum; Diagnosis, Differential; Female; Hemoperitoneum; Humans; Magnetic Resonance Imaging; Multimodal Imaging; Rupture, Spontaneous; Tomography, X-Ray Computed; Ultrasonography
PubMed: 32822245
DOI: 10.1259/bjr.20200383 -
American Journal of Obstetrics and... Apr 2024This study aimed to synthesize the existing evidence on perinatal outcomes after autologous cryopreserved ovarian tissue transplantation, concurrently identifying key... (Review)
Review
OBJECTIVE
This study aimed to synthesize the existing evidence on perinatal outcomes after autologous cryopreserved ovarian tissue transplantation, concurrently identifying key factors influencing these outcomes.
DATA SOURCES
A comprehensive search was performed on MEDLINE, Embase, and Cochrane Library databases to identify relevant studies on the effect of autologous cryopreserved ovarian tissue transplantation on perinatal outcomes from inception to October 22, 2023. Where there was missing information, the authors were contacted for updated data.
STUDY ELIGIBILITY CRITERIA
Observational studies, such as cohort studies, case series, and case reports that reported a live birth after autologous cryopreserved ovarian tissue transplantation, were considered eligible. Studies lacking data on women's demographic characteristics, autologous cryopreserved ovarian tissue transplantation procedure details, or perinatal outcomes were excluded. In addition, cases involving fresh or nonautologous transplantations and those addressing primary ovarian insufficiency were excluded.
METHODS
Two reviewers (M.E. and E.U.) independently performed the study selection, data extraction, and risk of bias assessment, and the results were then reviewed together. The PRISMA guidelines were followed, and the protocol was registered on PROSPERO (CRD42023469296).
RESULTS
This review included 58 studies composed of 122 women with 162 deliveries (154 singletons and 8 twins) after autologous cryopreserved ovarian tissue transplantation, resulting in 170 newborns. Of note, 83.6% of the women had a malignant disease. Moreover, most of these women (51.0%) were exposed to some form of chemotherapy before ovarian tissue cryopreservation. Of the 162 childbirths, 108 (66.7%) were conceived naturally, and 54 (33.3%) were conceived through assisted reproductive techniques. The birthweight of 88.5% of newborns was appropriate for gestational age, whereas 8.3% and 3.1% were small for gestational age and large for gestational age, respectively. The preterm birth rate was 9.4%, with the remaining being term deliveries. Hypertensive disorders of pregnancy were noted in 18.9% of women, including pregnancy-induced hypertension in 7.6%, preeclampsia in 9.4%, and hemolysis, elevated liver enzymes, and low platelet count in 1.9%. The incidences of gestational diabetes mellitus and preterm premature rupture of membranes were 3.8% for each condition. Neonatal anomalies were reported in 3 transplant recipients with 4 newborns: arthrogryposis, congenital cataract, and diaphragmatic hernia in a twin. Finally, among the recipients' characteristics, not receiving chemotherapy before ovarian tissue cryopreservation (odds ratio, 0.23; 95% confidence interval, 0.07-0.72; P=.012) and natural conception (odds ratio, 0.29; 95% confidence interval, 0.09-0.92; P=.035) were associated with a lower perinatal complication rate.
CONCLUSION
On the basis of low certainty evidence from observational studies, perinatal complication rates did not increase after autologous cryopreserved ovarian tissue transplantation compared with the general pregnant population, except for preeclampsia. This could be due to chemotherapy exposure, underlying medical conditions, and the common use of assisted reproductive techniques. Further larger studies are needed to explore the causes of increased preeclampsia incidence in autologous cryopreserved ovarian tissue transplantation pregnancies.
PubMed: 38621483
DOI: 10.1016/j.ajog.2024.04.012 -
Abdominal Radiology (New York) Jun 2024Gynecological emergencies can often present with a myriad of non-specific signs and symptoms, posing a diagnostic challenge to the clinician. They can be grossly divided... (Review)
Review
Gynecological emergencies can often present with a myriad of non-specific signs and symptoms, posing a diagnostic challenge to the clinician. They can be grossly divided into uterine or adnexal pathologies. Uterine pathologies can be secondary to intracavitary accumulation of blood [like in patients with transverse vaginal septum, Robert's uterus, accessory and cavitated uterine mass, unicornuate uterus with contralateral non-communicating functional horn], bleeding per vaginum [like in patients with retained products of conception, enhanced myometrial vascularity], pyometra [secondary to pelvic inflammatory disease, cervical obstruction secondary to benign and malignant causes] or complications of fibroids [like red degeneration, torsion of subserosal fibroid]. The adnexal pathologies can range from ectopic pregnancy in a urine pregnancy test (UPT) positive patient to haemorrhagic ovarian cyst, ovarian torsion, ruptured dermoid cyst and tubo-ovarian abscess in a UPT negative patient. Multimodality imaging including ultrasound (USG), computed tomography (CT) scan and magnetic resonance imaging (MRI) can narrow down the differentials and help in formulating an accurate diagnosis. The objective of this article is to familiarize the readers with multimodality imaging findings in common as well as uncommon acute gynecological emergencies and provide an algorithmic imaging approach for acute gynecological emergencies. USG is typically used as the first line diagnostic modality in diagnosis of acute gynecological emergencies. CT scan & MRI are helpful as a problem-solving tool in acute gynecological emergencies when USG findings are indeterminate.
PubMed: 38836883
DOI: 10.1007/s00261-024-04399-1 -
Seminars in Pediatric Surgery Oct 2023Performance of the appropriate operation is highly important to ensure that any patient with a suspected ovarian germ cell tumor receives optimal therapy that... (Review)
Review
Performance of the appropriate operation is highly important to ensure that any patient with a suspected ovarian germ cell tumor receives optimal therapy that prioritizes cure while simultaneoulsy minimizing risk of short and long-term toxicities of treatment. The following critical elements of any operative procedure performed for a suspected pediatric or adolescent ovarian germ cell tumor are reviewed: 1. Complete resection of the tumor via ipsilateral oophorectomy while avoiding tumor rupture and spillage, and 2. Performance of complete intraperitoneal staging at the time of initial tumor resection.
Topics: Adolescent; Child; Female; Humans; Neoplasm Staging; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms
PubMed: 38039829
DOI: 10.1016/j.sempedsurg.2023.151342 -
Radiology Case Reports Jan 2023Cryptorchidism is a relatively common and important clinical entity and can lead to an array of downstream complications if it is not corrected in a timely manner, most...
Cryptorchidism is a relatively common and important clinical entity and can lead to an array of downstream complications if it is not corrected in a timely manner, most notably with the development of testicular germ cell tumors. However, beyond the development of malignancy, there are other rare complications associated with cryptorchid testicular germ cell tumors which are more commonly seen in females with ovarian germ cell tumors, including torsion, rupture, and paraneoplastic syndromes. Presented is an instructive case (with literature review) of a patient who presented with NMDA encephalitis due to a torsed mixed germ cell tumor of an undescended testis, which subsequently ruptured leading to growing teratoma syndrome.
PubMed: 36345463
DOI: 10.1016/j.radcr.2022.10.022 -
Molecular Aspects of Medicine Apr 2021The development of the ovarian follicle to its culmination by ovulation is an essential element of fertility. The final stages of ovarian follicular growth are...
The development of the ovarian follicle to its culmination by ovulation is an essential element of fertility. The final stages of ovarian follicular growth are characterized by granulosa cell proliferation and differentiation, and steroid synthesis under the influence of follicle-stimulating hormone (FSH). The result is a population of granulosa cells poised to respond to the ovulatory surge of luteinizing hormone (LH). Members of the nuclear receptor superfamily of transcription factors play indispensable roles in the regulation of these events. The key regulators of the final stages of follicular growth that precede ovulation from this family include the estrogen receptor beta (ESR2) and the androgen receptor (AR), with additional roles for others, including steroidogenic factor-1 (SF-1) and liver receptor homolog-1 (LRH-1). Following the LH surge, the mural and cumulus granulosa cells undergo rapid changes that result in expansion of the cumulus layer, and a shift in ovarian steroid hormone biosynthesis from estradiol to progesterone production. The nuclear receptor best associated with these events is LRH-1. Inadequate cumulus expansion is also observed in the absence of AR and ESR2, but not the progesterone receptor (PGR). The terminal stages of ovulation are regulated by PGR, which increases the abundance of the proteases that are directly responsible for rupture. It further regulates the prostaglandins and cytokines associated with the inflammatory-like characteristics of ovulation. LRH-1 regulates PGR, and is also a key regulator of steroidogenesis, cellular proliferation, and cellular migration, and cytoskeletal remodeling. In summary, nuclear receptors are among the panoply of transcriptional regulators with roles in ovulation, and several are necessary for normal ovarian function.
Topics: Female; Follicle Stimulating Hormone; Granulosa Cells; Humans; Luteinizing Hormone; Ovarian Follicle; Ovulation
PubMed: 33288229
DOI: 10.1016/j.mam.2020.100937