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European Journal of Pediatric Surgery :... Feb 2024All types of cloacal malformations may be associated with anatomic variations of the external genitalia, including hypoplasia of the labia minora and enlarged...
Anatomical Variations of the External Genitalia in Posterior Cloaca: Clinical Consequences of Misdiagnosis-A Systematic Review of the Literature and the ARM-Net Consortium Experience.
PURPOSE
All types of cloacal malformations may be associated with anatomic variations of the external genitalia, including hypoplasia of the labia minora and enlarged clitoris; these variations could be even higher in posterior cloacas (PCs). If a careful physical examination is not performed, patients may be misdiagnosed with ambiguous genitalia (AG), leading to subsequent unnecessary testing, surgeries, or even wrong gender assignment. The aim was to analyze data of patients with PC within the ARM-Net registry, focusing on the description of the genitalia, gender assignment, and its consequences. Additionally, we investigated the presence of AG diagnosis in utero or at birth in patients with PC in the literature.
METHODS
The ARM-Net registry was scanned for PC cases and data on diagnosis were collected. A systematic literature search was conducted using the PubMed, EMbase, and Web-of-Science databases. Descriptive statistics was used to report data.
RESULTS
Nine patients with PC were identified in the ARM-Net registry. Five patients (55%) were diagnosed with AG, two (22%) were assigned as males and only two patients were correctly assigned as females and diagnosed with PC with respective variations of external genitalia. All patients diagnosed with AG had extensive blood testing including karyotype and hormonal studies. One of the patients who was diagnosed as a male, had surgery for pelvic cystic mass removal, which ultimately led to unaware salpingo-oophorectomy, hysterectomy, and vaginectomy. In the literature we identified 60 patients, 14 (23%) with AG, 1 with clitorolabial transposition and 1 with undeveloped vulva and vagina; 4 patients had normal anatomy. In 40 (67%) patients the anatomy of genitalia was not mentioned.
CONCLUSION
Patients with PC are at high risk of being diagnosed with AG or even assigned the wrong gender at birth. In our series two patients were assigned as males, and consequently one of them underwent a highly mutilating surgery. A thorough physical examination together with a high index of suspicion and laboratory workup are mandatory to identify these variations, avoiding further investigations, unnecessary surgeries, and parental stress.
PubMed: 38216143
DOI: 10.1055/a-2244-4551 -
Annals of Surgical Oncology May 2024Extended pelvic surgery with neurovascular or bony resections in gynecological oncology has significant impact on quality of life (QoL) and high morbidity. The objective...
BACKGROUND
Extended pelvic surgery with neurovascular or bony resections in gynecological oncology has significant impact on quality of life (QoL) and high morbidity. The objective of this systematic review was to provide an overview of QoL, morbidity and mortality following these procedures.
METHODS
The registered PROSPERO protocol included database-specific search strategies. Studies from 1966 onwards reporting on QoL after extended pelvic surgery with neurovascular or bony resections for gynecological cancer were considered eligible. All others were excluded. Study selection (Rayyan), data extraction, rating of evidence (GRADE) and risk of bias (ROBINS-I) were performed independently by two reviewers.
RESULTS
Of 349 identified records, 121 patients from 11 studies were included-one prospective study, seven retrospective studies, and three case reports. All studies were of very low quality and with an overall serious risk of bias. Primary tumor location was the cervix (n = 78, 48.9%), vulva (n = 30, 18.4%), uterus (n = 21, 12.9%), endometrium (n = 15, 9.2%), ovary (n = 8, 4.9%), (neo)vagina (n = 3, 1.8%), Gartner duct/paracolpium (n = 1, 0.6%), or synchronous tumors (n = 3, 1.8%), or were not reported (n = 4, 2.5%). Bony resections included the pelvic bone (n = 36), sacrum (n = 2), and transverse process of L5 (n = 1). Margins were negative in 70 patients and positive in 13 patients. Thirty-day mortality was 1.7% (2/121). Three studies used validated QoL questionnaires and seven used non-validated measurements; all reported acceptable QoL postoperatively.
CONCLUSIONS
In this highly selected patient group, mortality and QoL seem to be acceptable, with a high morbidity rate. This comprehensive study will help to inform eligible patients about the outcomes of extended pelvic surgery with neurovascular or bony resections. Future collaborative studies can enable the collection of QoL data in a validated, uniform manner.
Topics: Female; Humans; Quality of Life; Retrospective Studies; Prospective Studies; Pelvic Bones; Sacrum
PubMed: 38459419
DOI: 10.1245/s10434-023-14649-z -
Reproductive Sciences (Thousand Oaks,... Feb 2024The metastasis of a gynecological malignancy to the Bartholin gland is rare. We report the case of a 62-year-old patient who had undergone extensive treatment of...
The metastasis of a gynecological malignancy to the Bartholin gland is rare. We report the case of a 62-year-old patient who had undergone extensive treatment of metastatic ovarian cancer that involved the liver, spleen, and peritoneum. She presented with painful swelling of the left vulva. Clinical and sonographic examinations showed a solid tumor in loco typico of the Bartholin gland. Surgical excision was performed. The patient died 3 months after the diagnosis of this metastasis. We performed a systematic search of PubMed, which yielded 453 entries. We selected those with at least an abstract available in English that described metastatic lesions on the Bartholin gland (n = 5). The review showed that a variety of primary cancers (colorectal, medullary thyroid, breast cancer, and endometrial cancers) metastasize to this location. Some patients showed signs of visceral metastasis. Bartholin gland metastases appeared as initial and metachronous manifestations. Most patients were symptomatic, with painful swelling or abscess. Genetic alterations were mentioned in some cases. The main pathways of metastasis discussed were lymphatic, but the mechanism of such metastasis remains unclear. Surgical resection was the preferred treatment option. The literature review indicated that Bartholin gland metastasis of ovarian cancer is rare and associated with poor prognosis. Oncological reasons for vulvar pathologies should be taken into consideration in patients with metastases.
Topics: Humans; Female; Middle Aged; Bartholin's Glands; Ovarian Neoplasms; Breast Neoplasms; Gynecology
PubMed: 37794197
DOI: 10.1007/s43032-023-01373-y