The disturbed gonadal differentiation: dysgenesis and ovotesticular disorder of sex formation

2015 ◽  
Vol 61 (1) ◽  
pp. 31-35
Author(s):  
D A Morozov ◽  
N Yu Raygorodskaya ◽  
N V Bolotova ◽  
E S Pimenova ◽  
G N Maslyakova ◽  
...  

The objective of the present study was differential diagnostics between ovotestis and gonadal dysgenesis, the choice of the gender and the surgical strategy for the treatment of abnormal sexual glands. Diagnostics of the disturbances of gonadal differentiation (DGD) requires morphological verification. The ovotesticular gonad is characterized by the presence of a mature ovarian tissue and a dysgenetic testicular component. The authors describe the surgical separation of the ovotestis with preservation of the segments represented by the mature ovarian tissue. The presence of immature ovarian elements in the testicular gonad is one of the signs of its dysgenesis. Such cases need to be treated by the surgical removal of dysgenetic gonads.

Author(s):  
Juliana Gabriel Ribeiro de Andrade ◽  
Liliana Aparecida Lucci De Angelo Andrade ◽  
Gil Guerra-Junior ◽  
Andréa Trevas Maciel-Guerra

AbstractBackground:The 45,X/46,XY karyotype has been associated with mixed gonadal dysgenesis (MGD) and ovotesticular disorder of sex development (DSD). Our aim was to revise the diagnosis of ovotesticular DSD in two patients in the context of a retrospective study of 45,X/46,XY subjects with genital ambiguity.Case presentation:Patient 1 had a left streak gonad; the right one was considered an ovotestis. Patient 2 had a right testis; the left gonad was considered an ovary. Revision of the histological sections was performed. Both the “ovarian” part of the right gonad of patient 1 and the left “ovary” of patient 2 contained ovarian-type stroma with clusters of sex-cordlike structures and rare germ cells, compatible with undifferentiated gonadal tissue (UGT). Misdiagnosis of ovarian tissue in patients with 45,X/46,XY mosaicism or its variants could also be found in six published case reports.Conclusions:A distinction between 45,X/46,XY ovotesticular DSD and MGD should be made on past and future cases keeping in mind that UGT may be mistaken as ovarian tissue.


2019 ◽  
Vol 8 (3) ◽  
pp. 46-52
Author(s):  
L. M. Mikhaleva ◽  
A. A. Solomatina ◽  
M. Z. Argun ◽  
T. N. Khovanskaya ◽  
A. E. Biryukov

The aim of the study was to analyze the effect of surgical intervention on the status of the ovarian follicular apparatus in patients with benign ovarian cystic tumors (BCOTs) based on the results of morphometric studies.Material and methods. The authors have studied surgical material of 60 patients aged 18-47 years with BCOTs: serous cystadenoma (20 cases), serous papillary cystadenoma (7 cases), mucinous cystadenoma (12 cases), mature teratoma (23 cases). Microscopic slides of ovarian tissue stained with hematoxylin and eosin were obtained and morphological structure of BCOTs, presence of the intact ovarian tissue surrounding neoplasm and the density of follicles there were studied based on their qualitative characteristics (maturity, signs of degeneration).Results. It was found out that development of fibrosis in the wall of BCOTs significantly depends on the tumor histogenesis: the greatest thickness of fibrosis was detected in serous papillary cystadenomas, the smallest in the group of mature teratomas, the average values were determined in mucinous and serous cystadenomas of the ovaries. The influence of BCOTs on the ovarian reserve does not depend on its morphological structure, localization and diameter. The intact ovarian tissue is removed along with BCOTs during adnexectomy, ovarian resection and in 48.8% of cases – during cystectomy. During cystectomy, probability of the perifocal intact ovarian tissue presence is reduced due to the growth of fibrous tissue in this area. However, the negative impact of BCOTs on the ovarian reserve occurs not only due to surgical removal of the ovarian tissue, but also as a result of inhibition of folliculogenesis at the preoperative stage.Conclusion. BCOTs of the presented morphological groups negatively affect the follicular apparatus due to both – inhibition of folliculogenesis at the preoperative stage and loss of ovarian tissue during surgery. More pronounced perifocal proliferation of fibrous tissue was noted with serous papillary cystadenomas.


2021 ◽  
Vol 3 (59) ◽  
pp. 12-17
Author(s):  
Sergiu Beliniuc ◽  
◽  
Gabriela Motelica ◽  

Background. Coronectomy of the wisdom teeth is an alternative surgical procedure to tooth extraction, which aims to preserve the roots of the wisdom teeth in the dental socket after surgical separation of the crown, in order to avoid the injury of the inferior alveolar vascular-nervous bundle. Purpose. Minimizing the damage to the inferior alveolar nerve when extracting lower third molars caused by the intimate relationship between the nerve and the roots of the teeth, by using the technique of coronectomy, or intentional root retention. Material and Methods. Patients who reported to University Dental clinic during the period 2020–2021 for surgical removal of impacted L3M were screened for nerve–root relationship with OPG or CBCT. 15 patients underwent coronectomy as a procedure to remove the crown and upper third of the roots of a lower third molar to reduce the risk of damage to the inferior alveolar nerve. Results. 15 patients were enrolled in this study, with a total of 17 lower third molars. Sixteen sites healed primarily, but in 1 case the sockets on both sides opened and failed to close secondarily. In this case, the root fragments were later removed and found to be mobile. Conclusion. Coronectomies are safer to perform than complete extractions in situations in which the third molar is in close proximity to the mandibular canal. The technique appears to be associated with a low incidence of complications and the removal of remaining roots is required in around 6-7% of cases due to the mesial migration of the fragment and not any symptoms or reinfection.


2015 ◽  
Vol 61 (1) ◽  
pp. 57-60
Author(s):  
N V Severskaya ◽  
D Yu Semin ◽  
A A Ilyin ◽  
M I Ryzhenkova ◽  
I V Chebotareva

A case of isolated sarcoidosis of cervical lymph nodes is reported that was first erroneously interpreted as parathyroid adenoma. The patient was a 70 year-old woman presenting with hypercalcemia and elevated blood parathormone level. The ultrasound study showed the presence of a tissue mass measuring 1.5 cm in the projection of the inferior right parathyroid gland. The scintigraphic study with 99mTc MIBI demonstrated the accumulation of the radiopharmacological agent in the tissue mass that was identified as an adenoma of the parathyroid gland. The surgical removal of this neoplasm together with the adjacent lymph nodes resulted in the normalization of the blood calcium and parathormone levels. The histological study revealed sarcoidosis of lymph nodes in the absence of lesions in other organs. A literature review of the problem pertaining to hypercalcemia associated with sarcoidosis of cervical lymph nodes is presented with special reference to the difficulties encountered in differential diagnostics of this condition.


2017 ◽  
Vol 63 (3) ◽  
pp. 201-203
Author(s):  
Nadezda Y. Raygorodskaya ◽  
Nina V. Bolotova ◽  
Danil A. Jarkov ◽  
Tatyana V. Palatova ◽  
Natalya S. Dorovskaya

A 46,XY ovotesticular disorder of sexual development is a rare variant of pathological gonadal differentiation. A 15-month-old patient had ambiguous external genitalia, no palpable gonads, and the 46,XY karyotype. The uterus was detected by imaging of the lesser pelvis. Gonads resided on the fallopian tubes and macroscopically resembled ovotestes: each gonad consisted of two compartments separated by a connective tissue interlayer. Histological examination showed that one gonadal portion consisted for ovarian tissue, was differentiated into the cortical and medullary matter, and contained primordial follicles with pronounced dystrophic changes. The remaining portions consisted of immature tubular epithelium with proliferative cellular changes. The decision about bringing up as a female with possible adaptation during puberty was justified in this case. The surgical approach was selected on the basis of histological examination and a decision on performing gonadectomy was made.


AYUSHDHARA ◽  
2020 ◽  
pp. 2393-2399
Author(s):  
Arunava Nath* ◽  
Deb Kumar Palit ◽  
Nivedita Kundu

Ovarian enlargements, cystic or solid, may occur at any age. Functional and inflammatory enlargements of the ovary develop almost exclusively during the childbearing years. They may be asymptomatic or produce local discomfort, menstrual disturbances, infertility, or in rare cases cause acute symptoms due to complications like haemorrhage, rupture or torsion. A case on dysmenorrhoea along with right ovarian cyst measuring about 52x45 mm has been presented which was treated with constitutional homoeopathic medicine Lycopodium clavatum. Literature review suggested spontaneous regression of cysts within 3 months with oral combined pills but in the presented case the subject had been suffering for last one year and was under hormonal treatment with no favourable results. In the case of simple cysts more than 5 cm in diameter and complex cysts, surgical removal of the mass is most often recommended in an attempt to preserve viable ovarian tissue. With homoeopathic approach the treatment lasted for nearly one year with gradual reduction in pain intensity and sonographically no detectable abnormality was noted after treatment.


2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 15064-15064
Author(s):  
T. Lee ◽  
J. Jung ◽  
J. Kim ◽  
J. Kim ◽  
N. Park ◽  
...  

15064 Background: The treatment of endometrial cancer involves surgical removal of the ovary; this elimination induces an abrupt menopause and may deteriorate the qualities of life. Therefore, ovarian preservation may be a consideration for premenopausal women. Our main objectives are to examine the occurrence of ovarian metastasis or synchronous malignancy and to evaluate the feasibility of ovarian preservation in patients with early stage endometrial carcinoma. Methods: We reviewed the medical records of 259 patients undergoing surgical treatment for endometrial cancer at a single institute from 1992 to 2004. Results: Among the 224 patients with endometrial adenocarcinoma who had undergone ovarian removal, cancer in ovarian tissue was detected in 21 cases (9.4%, 14 ovarian metastasis, 7 synchronous cancer). Synchronous ovarian cancer showed abnormal gross finding in all 7 cases. Thirteen cases of ovarian metastasis were high grade lesion in preoperative evaluation, or showed intraoperative peritoneal seeding or abnormal gross lesion around adnexa. In 35 patients, grossly normal ovary was saved selectively in compliance with patients’ need (19 bilateral, 16 unilateral). Thirty-one of 35 (89%) were under 45 years and mostly showed early stage (Ia, 24; Ib, 7; Ic, 1; IIa, 1; IIb, 2). Pre-operative MRI was available in 30 cases, and none of them showed findings suggesting tumor extension outside of uterus. In 2 cases of IIb, postoperative radiation therapy was done. There was no recurrence or death in all cases of ovarian preservation except one in which a patient died of sepsis caused by postoperative bowel perforation (median duration of follow-up, 76 mon.; range 3∼121). Conclusions: Ovarian preservation can be cautiously performed, preceded by a thorough preoperative and intraoperative assessment of the adnexa in young women with endometrial carcinoma. The patients who desire ovarian preservation should be counseled regarding the rate of ovarian metastasis or synchronous malignancy. No significant financial relationships to disclose.


2012 ◽  
Vol 153 (8) ◽  
pp. 303-307 ◽  
Author(s):  
László Ságodi ◽  
Janka Jakab ◽  
Ákos Kiss ◽  
Erzsébet Ladányi ◽  
Erzsébet Balogh ◽  
...  

The authors report a case of a dysgenetic male pseudohermaphroditism with a 45,X/46,XY karyotype in a mosaic form, which was diagnosed in an infant. The one-week-old infant was evaluated because of proximal hypospadias and retention of the right testis. The results of hormonal tests were the followings: serum FSH 5.2 mU/ml; LH: 2.0 mU/ml; testosterone: 144.3 ng/dl; androstendione: 0.42 µg/l; 17-hydroxyprogesterone: 1.12 ng/ml. Chromosomal analysis revealed 45,X/46,XY karyotype. Fluorescent in vitro hybridization showed that 51% of the lymphocytes had the Y chromosome and the SRY gene. Analysis of the SRY showed no deletion in the AZF a,b,c regions. Pelvic magnetic resonance imaging indicated the presence of vagina between the bladder and the rectum, and it showed a mass measuring 15×8 mm in the right inguinal canal as well as an oval gonadal mass with a size of 13×7 mm in the left scrotum. During surgical intervention, performed at the age of one, the right gonad was removed and biopsy of the scrotal testis was performed. Histological examination revealed dysgenetic testis in both sides. The authors emphasize the necessity of cytogenetic and endocrinological investigations of newborns with perineoscrotal hypospadia and bilateral or unilateral maldescent testes immediately after birth. Surgical removal of the dysgenetic testicular tissue located in the abdominal cavity and its histological evaluation provides separation of mixed gonadal dysgenesis, dysgenetic male pseudohermaphroditism, bilateral gonadal dysgenesis and ovotestis in the 45,X/46,XY mosaic cases. An accurate evaluation is necessary for a correct sex assignment and for surgical intervention to prevent neoplastic degeneration of the dysgenetic gonad. Orv. Hetil., 2012, 153, 303–307.


2005 ◽  
Vol 56 (suppl_4) ◽  
pp. ONS-337-ONS-343 ◽  
Author(s):  
Ricardo Ramina ◽  
Joao J. Maniglia ◽  
Jorge R. Paschoal ◽  
Yvens B. Fernandes ◽  
Mauricio Coelho Neto ◽  
...  

Abstract OBJECTIVE: The surgical removal of a jugular foramen (JF) tumor presents the neurosurgeon with a complex management problem that requires an understanding of the natural history, diagnosis, surgical approaches, and postoperative complications. Cerebrospinal fluid (CSF) leakage is one of the most common complications of this surgery. Different surgical approaches and management concepts to avoid this complication have been described, mainly in the ear, nose, and throat literature. The purpose of this study was to review the results of CSF leakage prevention in a series of 66 patients with JF tumors operated on by a multidisciplinary cranial base team using a new technique for cranial base reconstruction. METHODS: We retrospectively studied 66 patients who had JF tumors with intracranial extension and who underwent surgical treatment in our institutions from January 1987 to December 2001. Paragangliomas were the most frequent lesions, followed by schwannomas and meningiomas. All patients were operated on using the same multidisciplinary surgical approach (neurosurgeons and ear, nose, and throat surgeons). A surgical strategy for reconstruction of the cranial base using vascularized flaps was carried out. The closure of the surgical wound was performed in three layers. A specially developed myofascial flap (temporalis fascia, cervical fascia, and sternocleidomastoid muscle) associated to the inferior rotation of the posterior portion of the temporalis muscle was used to reconstruct the cranial base with vascularized flaps. RESULTS: In this series of 66 patients, postoperative CSF leakage developed in three cases. These patients presented with very large or recurrent tumors, and the postoperative CSF fistulae were surgically closed. The cosmetic result obtained with this reconstruction was classified as excellent or good in all patients. CONCLUSION: Our results compare favorably with those reported in the literature. The surgical strategy used for cranial base reconstruction presented in this article has several advantages over the current surgical techniques used in cases of JF tumors.


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