Manupriya Sharma

Angioleiomyoma Uterus in an Adolescent Girl: A Highly Unusual Presentation

BACKGROUNDnUterine angioleiomyoma is a very rare gynecologic tumor; only a few such cases have been reported to date and we have found no such tumor reported in an adolescent girl.nnnCASEnWe report the case of a uterine angioleiomyoma in an adolescent girl. It presented as a huge abdomino-pelvic mass. The girl had severe menorrhagia and severe anemia. Intra-operatively no distinct planes were found between myoma and myometrium. Due to severe hemorrhage, she underwent sub-total abdominal hysterectomy.nnnSUMMARY AND CONCLUSIONnUterine angioleiomyoma is an extremely rare tumor since only 16 such cases have been reported to date. Its appearance in an adolescent girl seems to be the first case of its kind. So it is being reported not only to familiarize the managing physicians with the possibility of such a tumor and its variable presentation, but also to highlight the need for inclusion of this tumor in WHO classification of tumors of the female genital tract.

Rupture of Uterus in Mid Trimester of Pregnancy: A Case Series & Review of Literature

With the increasing rates of cesarean section (CS) worldwide, pregnant women with previous CS are also rising at an alarming rate. This is leading to increase in previously rare complications of pregnancy e.g. cesarean scar pregnancy, placenta accreta and rupture uterus. One such complication is mid trimester rupture of uterus, which previously used to be a rare possibility. Now a day’s more and more pregnant women are presenting in shock in mid trimester of pregnancy. Hence, this case series is being reported to highlight this complication of pregnancy and to remind managing obstetricians and sonologists regarding these rare possibilities which should be kept in mind while doing routine sonographic assessment of pregnant women, so as to prevent catastrophe of rupture uterus.

Paucibacillary Tubercular Vulval Ulcer in a Sexually Inactive Pubertal Girl: Role of Therapeutic Trial

BACKGROUNDnGenital ulcers in adolescent girls present a complex diagnostic dilemma. Of all the causes of painful vulval ulcer in a virgin girl, tuberculosis is an extremely rare possibility with no case reported in literature.nnnCASEnWe are reporting a case of tubercular vulval ulcer in a sexually inactive pubertal girl. She had a close contact with active tuberculosis, positive tuberculin skin test, and chronic granulomatous inflammation on vulval biopsy. Therapeutic trial was offered and on follow up visit after 4 weeks, lesion had completely resolved.nnnSUMMARY AND CONCLUSIONnUnlike previous case reports that describe tubercular vulval ulcers in sexually active or post menopausal women our patient was sexually inactive. In endemic areas tuberculosis should be considered in differential diagnosis of vulval ulcers irrespective of sexual history of the patient.

Pre‐pubertal isolated plexiform neurofibroma of labium majus without clitoral involvement

Sir, Neurofibromas are tumors of neural crest origin with protean manifestations which can affect virtually every organ and system. Isolated involvement of the female genital organs by this disease is rare and clitoral hypertrophy is the most common presentation. Genital neurofibromas usually enlarge in size and become symptomatic during puberty and pregnancy; however, we report an isolated plexiform neurofibroma in one labium majus without clitoral involvement and with documented rapid growth in a pre-pubertal girl. A 9-year-old girl presented with a swelling in the vulval region present since birth and with progressive painless enlargement for the last two years. The mass remained completely asymptomatic till the age of six years when it started to increase in size, causing discomfort during walking. The girl was 112 cm tall, breasts were Tanner’s stage I and pubic and axillary hair absent. Multiple (more than six) café-aulait spots measuring >15 mm were present over the abdomen, back and buttocks (Figure 1). A 7.2 × 5.4-cm sized irregular growth was noted in the left labium majus leading to disfigurement. The clitoris was unremarkable. The growth was firm, not tender, irreducible, with ill-defined margins and without vaginal extension. Ultrasound showed a predominantly hypo-echoic lesion with septae without any pelvic organ abnormality. Fine needle aspiration cytology suggested a benign spindle cell tumor. The mass was excised and histopathology revealed plexiform neurofibromatosis (Figure 1). Neurofibromatosis-1 (NF1) is an autosomal dominant neurocutaneous syndrome with a reported incidence of 1 in 3000 live births. It is a multisystem disorder which may affect virtually every structure of the body; however, involvement of the external genitalia

Pelvic Organ Prolapse during Pregnancy: A Case Series and Review of Literature

Pelvic organ prolapse during pregnancy is extremely rare. Limited cases (less than 30) have been reported since 1980. From a very benign presentation of heaviness in perineum, it can present as uterine rupture with fetal and maternal mortality. No standard guidelines of care have been established for this rare presentation. There is gross variation in management ranging from conservative measures, laparoscopic surgery to cesarean section followed by peri-partum hysterectomy and abdominal sacral colpopexy. This case series report five cases of pelvic organ prolapse during pregnancy and outlines an approach of watchful expectancy with favorable maternal & fetal outcomes.

Enterobius vermicularis infestation leading to meckel's diverticulitis in an adolescent boy: An extremely rare presentation

Enterobius vermicularis is an intestinal nematode commonly affecting children worldwide. Its transmission is by feco-oral route. Meckels diverticulitis due to E. vermicularis infestation is an extremely rare presentation. An 11-year-old boy presented with acute abdomen. During surgery inflamed Meckels diverticulum (M.D) was seen. Histopathology examination of specimen revealed E. vermicularis. Till date, only one case of E. vermicularis infestation of M.D is reported around five decades ago. This histopathological confirmation is extremely important as the required treatment (Mebendazole) of the infected case along with household contacts can prevent the spread of infection and may avoid surgery in known contacts.

Pediatric Lymphadenopathy: Cytological Diagnosis Over a Period of Two Years in a Rural Teaching Hospital

Background: Lymphadenopathy (LAP) is a common clinical presentation seen in pediatric patients attending the outpatient department. The etiology of LAP varies from a benign process to malignancy. Fine needle aspiration cytology (FNAC) is a very rapid and easy to perform diagnostic test. Majority of cases can be diagnosed and managed on FNAC without need of histopathology. The aim of this study was to evaluate the role of FNAC in diagnosis of superficial LAP in pediatric population. nMethods: This retrospective study was carried out over a period of two years from January 2015 to December 2016 in the department of Pathology, Dr. RPGMC, Tanda, Himachal Pradesh, India. All cases of superficial LAP seen in pediatric population where FNAC was performed were included in the study. nResults: A total of 357 lymph nodes were aspirated in pediatric population during the study period. Majority of cases (98%) were benign. The results were categorized into three broad categories as reactive lymphadenitis (296; 82%), granulomatous lymphadenitis (56; 16%) and malignancy (05; 02%). The five cases of malignancies identified in this population comprised of two cases each of Hodgkin’s Lymphoma (HL) and Non-Hodgkin’s Lymphoma (NHL). One case was diagnosed as metastasis from papillary carcinoma thyroid. nConclusion: FNAC should be considered as a first line investigation for evaluating the peripheral LAP in pediatric population. The main etiology of LAP is reactive lymphadenitis. Majority of these cases can be diagnosed and managed on FNAC alone. FNAC can play a key role in triage of appropriate cases, so that these patients may be further investigated to make a diagnosis or for referral to higher centers.

Haematological profile of patients with mixed-phenotype acute leukaemia from a tertiary care centre of north India

Background & objectives: Mixed-phenotype acute leukaemia (MPAL) is a rare neoplasm with no definite treatment protocols and a distinctly poor outcome. Advancement in polychromatic flow cytometry has made its identification easier. This prospective study was designed to identify cases of MPAL and study their clinical presentation and haematological profile in a tertiary care hospital in north India. Methods: Ethylenediaminetetraacetic acid (EDTA)-anticoagulated bone marrow aspirate samples of patients diagnosed as acute leukaemia (AL) on the basis of morphology were utilized for immunophenotyping. A comprehensive panel of fluorochrome-labelled monoclonal antibodies targeting myeloid, B-cell, T-cell and immaturity markers was utilized. The patients diagnosed to have MPAL, on the basis of the World Health Organization 2008 classification, were selected for further analyses. Results: There were 15 (2.99%) patients with MPAL of the total 501 cases of AL. Seven were children, all males and mean age of 5.08±3.88 yr. Eight were adults, male:female=6:2 and mean age of 21.43±5.74 yr. Eight were diagnosed as B/myeloid and seven were T/myeloid. No association was observed between age and immunophenotype of MPAL. On morphology, 11 were diagnosed as AML and four as ALL, and no specific morphology of blasts was predictive of a MPAL. Interpretation & conclusions: MPAL appeared to be a rare neoplasm (2.99% of AL cases). A comprehensive primary panel of monoclonal antibodies should be used to identify this neoplasm known to have a poor outcome.

Characterization of immunophenotypic aberrancies in adult and childhood acute lymphoblastic leukemia: A study from Northern India

BACKGROUNDnIdentification of aberrant antigen expression is important in characterizing neoplastic population among non.neoplastic bone marrow counterparts and further in the detection of minimal residual disease. (MRD). Flow cytometry (FCM) is an important tool in identifying aberrant phenotypes. Incidence of aberrant phenotypes varies considerably in independent studies and its association with prognostic factors is still debatable.nnnAIMnTo identify the prevalence of aberrant phenotypes on immunophenotyping in a large series of de novo acute lymphoblastic leukemia (ALL) and to evaluate any association with initial clinical and hematological features.nnnMATERIALS AND METHODSnIn the current study, 303 patients of de novo ALL were included from the Department of Hematology, PGIMER, Chandigarh during the time period (July 2010 to June 2012). The immunophenotype of all cases of ALL was studied using FCM.nnnRESULTSnAberrant myeloid antigen expression was seen in 42.5% cases. Most frequent aberrant myeloid antigen was CD13 (32.2% cases), followed by CD33 (27.2% cases) and CD117 (18.5% cases). The expression of CD117 was relatively frequent in comparison to earlier reports which describe its rare expression. Adult T- ALL showed higher expression of CD33 and CD117 than pediatric T-ALL (P = 0.032 and 0.043, respectively). Myeloid antigen expression in ALL was associated with lower WBC count (P < 0.05) and lower number of peripheral blasts (P < 0.05). Expression of CD34 was higher in My + ALL group (P < 0.05) than My- ALL group.nnnCONCLUSIONnIn summary, CD117 is a relatively frequently expressed myeloid marker contrary to earlier reports which describes its rare expression. Pediatric and adult ALL cases with low blast count and CD34 positivity are more likely to express aberrant myeloid markers. Current study also supports that myeloid antigen expression in both adult and pediatric ALL is not associated with adverse presenting clinical and biological features.

A vaginal foreign body presenting as vesico-vaginal fistulae in a pre-pubertal girl: A case report and review of the literature

Background: A neglected foreign body in a vagina leading to vesico-vaginal fistulae in young girls is a very rare presentation. As such, it poses significant therapeutic challenges in its management. Case presentation: A 10-year old pre-pubertal girl presented with malodorous vaginal discharge and continuous dribbling of urine per vaginum. Two separate stones, one each in the vagina and the urinary bladder, with concomitant vesico-vaginal fistulae (VVF) were diagnosed. The history of a foreign body (the cap of a nail paint bottle) inserted into the vagina, while playing, about two years back was elicited. She underwent abdomino-vaginal repair with satisfactory outcome. Conclusion: Vaginal foreign bodies leading to stone formation and subsequent VVF are a rare presentation. Till date 12 such cases have been reported. This case is being reported to highlight this rare cause of VVF in young girls and to develop consensus regarding its management. The procedure of management here involved first removal of the vaginal foreign body and then prolonged catheterization followed by subsequent repair of VVF after 6-8 weeks. However, individualization of such cases may be required depending upon specific characteristics (e.g., large size in index case).