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Warning: The NCBI website requires JavaScript to operate. An unusual case of vaginal bleeding in an 18-year-old femaleSalim Akhtar1Department of Medicine, Roswell Park Cancer Institute, University at Buffalo, Buffalo, NY 14209 USA Kedar K. Deodhar2Tata Memorial Hospital, Mumbai, India Anupurva Dutta2 Memorial Hospital, Mumbai, India Farhat Jabeen3Government Medical College, Srinagar, Jammu Although it is known that they occur anywhere in the body, they rarely affect the female genital tract. Here we present a case of an 18-year-old female who presented us with abnormal vaginal bleeding and found herself with a non-secret vaginal paraganglioma. Case History An 18-year-old female presented episodes of increased vaginal bleeding over a period of 2 months. He did not complain of bleeding from any other site and had no prior or family history of coagulopathy or hemorrhagic disorders. When examining, his vital signs were normal, but he found himself pale. Masses didn't get palpated. The patient was not sexually active and had an intact hymen, so a complete pelvic examination could not be performed. Laboratory research revealed a hemoglobin level of 5.5 g/dL. The ultrasound (USG) of the pelvis and abdomen was performed and was not noticeable. The patient received blood transfusions. Magnetic resonance (MRI) of the pelvis was ordered showing a polypoid heterogeneous lesion within the vaginal cavity of about 3 Γ 1.6 cm (Fig. ). No extension was observed in the cervix or surrounding tissues. A biopsy of the lesion was taken and suggestive characteristics of a well-differentiated neuroendocrine tumor were revealed, with scant mytotic activity and mild ulceration of excessive mucosa. CT scan (CT) of the chest and abdomen was not noticeable, without evidence of metastases or primary in other places. Orine catecholamines and fractionated metanephens were also normal. The patient was taken for surgery, and the mass was completely enucleated, blocked. The histologic sections of the mass showed tumor cells arranged in nests, separated by thin fibrous septa with granular cytoplasm and minimal nuclear pleomorphism (Fig. a, b). In immunohistochemicals (IHC), the tumor was positive for synaptophysis, chromogranine and negative for demin, CD163 and AE1/AE3, a cell epithelial marker (Fig. c, d). A low score of Mib-1 was reported from 3 to4%. A diagnosis of non-functional vaginal paraganglioma was made. Magnetic resonance pelvis showing a hyperintense mass on the back wall of the vagina extending to corresponding fornix in images with T2 weight (at sagittal, b axial) a The normal squamous epithelial lining is seen (golden arrow) with lesion that shows a tumor pattern of anity (thinning arrow) and stipated tumor nuclei (Γ40). b Increase magnification by displaying annihilous pattern with relatively regular nuclei with minimal pleomorphism (Γ200). c The tumor's immunohistochemistry shows the negative AE1/AE3 marker in the tumor. The internal control of the squamous squamous epithelium is positive (Γ40). d Positive tumor ink for chromogranine (Γ200)The patient was put in follow-up and has been asymptomatic for the last 5 months. DiscussionParagangliomas (PGL) are rare neuroendocrine tumors of paraganglya and are associated with the sympathetic and parasympathetic system. They can be found in virtually every place where normal paraganglya is known, with tumors that arise from adrenal medulla traditionally known as pheochromocytomas []. As such, they are rarely found in the female genital tract, with only 6 reported cases of vaginal paraganglioma in the literature [].Clinically, the presentation of paragangliomas varies depending on the location and secret nature of the tumor among others. PGL that secret catecholamines often have sympathetic symptoms such as headaches, hypertension and arrhythmias. Symptoms often get worse in the manipulation of the injury, a feature that has important implications in the preoperative care of these patients []. The PGL that occurs outside the adrenal gland is usually associated with the parasympathetic and non-secret catecholamine system []. Vaginal paragangliomas have certain unique characteristics. Of the 6 cases reported above, half is presented with abnormal vaginal bleeding and/or a vaginal mass (table ). Patients may also have sympathetic symptoms during intercourse or biopsy/surgery. In one of the cases reported above, during the excision of what seemed to be an inactive mass, the patient suddenly landed in a hypertensive crisis, suggesting that even previous non-secret lesions can release catecholains during manipulation. Table 1Vaginal paragangliomas reported in literatureReferences AgeSecretary Presentation Treatment Cai et al. []17Secred vaginal bleeding; Surgical excision Mannelli et al. []65No secret pelvic mass embolization of uterine arteries; surgical excision Shen et al.38SecretoryVaginal mass; HTN; palpitations Surgical excisionBrustmann33No-secretVaginal bleedingExterior excisionHassan et al.24SecretoryMedical matter Parkes et al.11Non-secretory Vaginal bleeding HTN hypertension, MI myocardial infarction Establishing a diagnosis of preoperative vaginal paraganglioma can be difficult. Of 7 cases, 3 had symptoms of excess catecholamine. A blood or urine test to determine metanephens or noradrenaline may be useful in such patients. Radiological characteristics cannot distinguish a vaginal paraganglioma from other more common lesions on the vaginal wall as a smooth muscle tumor []. The pelvic USG can be undeniable, as in our case. A CT scan or MRI scan can help demarcate the size and extent of the injury. In addition, improved contrast computed tomography can show greater vascularity of the mass by causing less invasive strategies such as the embolization of the uterina artery before biopsy or excision. An FDG-PET/CT analysis can reveal increased absorption in the lesion and help identify metastatic lesions. Histopathology is essential for a definitive diagnosis. Histologically, lesions resemble paragangliomas in other places with cells often arranged in a characteristic nesting pattern (zellballen) with prominent vascular cutting as spaces between nests []. In immunohistochemicals, positivity for chromogranine, sinaptophysis, specific neuron enolase and S-100 has been systematically observed. The ultrastructural examination can reveal neurosecretary granules. Surgical excision is the definitive treatment for a vaginal paraganglioma. In one case, the embolization of the uterine artery was performed before the excision of a vascular mass. Preoperative alpha block therapy is regularly practiced []. A diagnosis of vaginal paraganglioma generally maintains a good prognosis. All reported cases had a non-remarkable follow-up period, without metastasis or dissemination reports. In conclusion, vaginal paragangliomas are rare tumors of the vaginal tract. A diagnosis can be considered in a patient with abnormal vaginal bleeding, symptoms of sympathetic overactivity during intercourse or pelvic examination, with greater vascularity in the CT scan. Appropriate preoperative care can prevent complications such as hypertensive or arrhythmias during surgery or biopsy. BiographyOthman Salim Akhtar, M.D. completed his training as a pathologist at SKIMS, Srinagar, before moving to the Weill Cornell Medical Center in New York City where he joined the Prof's lab. Ari Melnick, conducting lymphoma research for 1 year. He returned to Hakim Sanaullah Cancer Center, a rural cancer center in North Kashmir, for a short period of time to help establish diagnostic facilities there, before joining as a resident physician at Buffalo University, Buffalo, NY, USA. Compliance with ethical standards Conflict of interest The authors do not report any conflict of interest. Informed consentThe informed consent was obtained from the patient to prepare this manuscript. No personal information is shared in the description of this case, and no experiments were performed in the patient. FootnotesOthman Salim Akhtar is a resident at the University of Buffalo; Kedar K. Deodhar is a professor at the Department of Pathology at the Tata Memorial Hospital; Anupurva Dutta is a senior resident, Department of Pathology at Tata Memorial Hospital; Farhat Jabeen is an associate professor, Department of Obstetrics and Gynaecology at the Government Medical College; Shad Salimtar , 8600 Rockville Pike, Bethesda MD, 20894 USA
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