|Year : 2021 | Volume
| Issue : 4 | Page : 262-264
Hemoperitoneum due to ruptured botryoid sarcoma of the uterus in young girl
Rajendran Ramaswamy, Elsayed Ali, Saif Saeed Ghalib, Ghazi Mukattash
Department of Pediatric Surgery, Maternity and Children's Hospital (Under Ministry of Health), Najran, Saudi Arabia
|Date of Submission||29-Apr-2020|
|Date of Decision||07-Jun-2020|
|Date of Acceptance||07-Jul-2020|
|Date of Web Publication||12-Jul-2021|
Dr. Rajendran Ramaswamy
Department of Pediatric Surgery, Maternity and Children's Hospital (Under Ministry of Health), PB No: 3600, Najran
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Five-year-old girl presented with lower abdominal pain, pelvi-abdominal mass, and generalized abdominal tenderness. Ultrasound and computed tomography scans diagnosed heterogeneous pelvi-abdominal soft-tissue mass and a large amount of free peritoneal fluid. Laparotomy revealed hemoperitoneum and ruptured tumor at the posterior uterine wall. Histopathology report was botryoid rhabdomyosarcoma (BRMS). This case is unique due to ruptured BRMS of the uterus in early childhood, with no vaginal bleeding.
Keywords: Botryoid rhabdomyosarcoma, hemoperitoneum, ruptured rhabdomyosarcoma, uterine corpus tumor
|How to cite this article:|
Ramaswamy R, Ali E, Ghalib SS, Mukattash G. Hemoperitoneum due to ruptured botryoid sarcoma of the uterus in young girl. J Indian Assoc Pediatr Surg 2021;26:262-4
|How to cite this URL:|
Ramaswamy R, Ali E, Ghalib SS, Mukattash G. Hemoperitoneum due to ruptured botryoid sarcoma of the uterus in young girl. J Indian Assoc Pediatr Surg [serial online] 2021 [cited 2021 Jul 30];26:262-4. Available from: https://www.jiaps.com/text.asp?2021/26/4/262/321190
| Introduction|| |
Botryoid variety of embryonal rhabdomyosarcoma (ERMS) is found in hollow viscus such as the urinary bladder, vagina, and biliary tree. One of the least common sites for botryoid rhabdomyosarcoma (BRMS) in the genitourinary tract is the uterus. Intergroup rhabdomyosarcoma study (IRS) reported that rhabdomyosarcoma (RMS) usually arises from the vagina in infancy and early childhood, whereas that of the uterus is more often seen in adolescence. Vaginal bleeding, discharge, or fullness from the mass is commonly seen in botryoid sarcoma of the vagina or uterus. Clinically, a case of acute peritonitis and a pelvic mass proved to be ruptured uterine BRMS in the young child is reported herewith.
| Case Report|| |
A 5-year-old girl presented with lower abdominal pain and mass thereof 2 weeks, poor appetite and constipation for 3D and vomiting of 1D duration. She was found pale with heart rate 110/mt, respiration 30/mt, and blood pressure 115/85 mmHg. Her abdomen was not distended, but with generalized tenderness more on the right side, rebound tenderness in the right lower quadrant, and a firm mass in hypogastrium. The lower border of the mass was not palpable. Blood or mass was not seen from the vagina. Her hemoglobin level was 8.2 g/dl and hematocrit 24.3%. White-cell and platelet counts, coagulation profile, renal, and liver function tests were normal. Plain abdominal skiagram revealed haziness of the pelvis. Ultrasound scan revealed a heterogeneous pelvic mass, large amount of abdominal free-fluid, and right hydronephrosis Grade II. Contrast-enhanced computed tomography scan diagnosed pelviabdominal soft-tissue mass (size 9 cm × 11 cm) of different densities, large amounts of free peritoneal fluid, and right hydronephrosis [Figure 1]. Because of the clinical features of peritonitis, emergency laparotomy was done after blood transfusion. Laparotomy revealed hemoperitoneum. About 450 ml of blood was emptied. The uterus was found enlarged [Figure 2]. A big blood-clot protruding from the posterior uterine wall when removed, ruptured whitish friable uterine tumor came into view. The uterus was not movable. The ovaries, uterine tubes, liver, spleen, and intestines were normal. There was no lymph node enlargement found. Fragments of the tumor were taken for histopathologic examination (HPE), drains inserted and laparotomy closed. HPE and immunohistochemistry report was diagnostic of the botryoid variant of ERMS. The pretreatment stage of the tumor was obviously T2bN0Mx and clinical group Stage III. The child was referred to the pediatric oncology center for management.
|Figure 1: Contrast-enhanced computed tomography scan of the abdomen (coronal section), showing pelvi-abdominal heterogeneous soft-tissue mass and the large amount of free peritoneal-fluid|
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|Figure 2: Peroperative picture showing enlarged uterus with a ruptured tumor in the posterior surface of the uterus, normal left Fallopian tube More Details, and left ovary and hemoperitoneum|
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| Discussion|| |
Botryoid RMS accounts for about 10% of all RMS cases and arises under the mucosal surface of hollow organs such as the vagina, uterine cervix, bladder, and biliary tree. Vaginal tumors are five times more common than cervical tumors. BRMS of the vagina usually occurs in infancy and early childhood. RMS of the uterine cervix is extremely rare. BRMS of the uterine corpus is still rarer in children and its clinicopathological features are not clear. BRMS typically appears as a polypoid or grape-like mass.
The peak incidence of BRMS of the cervix is in the second half of adolescence and extremely rare in young childhood. Behtash et al. reviewed 19 cases, Gruessner et al. 20 cases and Dehner et al. reviewed 14 cases of BRMS of the cervix. The maximum incidence in all these studies was in 14 years–18 years age group and only a small minority in <10 years age. Vaginal bleeding was the symptom in almost all patients, followed by leucorrhea and mass from vaginal introitus. Other authors also reported similar observations.,, Seventy-five percent–91% of the patients had IRS Group I disease. ERMS of the uterine corpus is a rare and invasive tumor that is mostly seen in women of 20 years–45 years age range (reproductive phase)., The symptoms and signs of uterine ERMS may include abnormal vaginal discharge and vaginal bleeding, followed by uterine mass, abdominal discomfort, or pain. Primary ERMS of the uterine corpus is very rare in prepubertal girls. There was a report of 18-month-old girl presented with abdominopelvic mass, bleeding per vagina, and a mass through the introitus. This was proved as BRMS involving the uterus, cervix, and vagina. Another 10-year-old girl with a history of cystic nephroma treated in infancy, presented with vaginal bleeding of 3 weeks. She was found to have a uterine mass, which histopathologically was BRMS. Molecular genetic sequencing of the tumor sample discovered a DICER1 gene mutation. DICER1 gene, located on chromosome 14, position q32.13, encodes the endoribonuclease enzyme Dicer, concerned with micro-RNA biogenesis. Dicer1 germline mutations increase the risk of developing uncommon tumors. Our index case is 5-year-old patient with BRMS of uterine corpus involving cervix also. This patient has presented with abdominal pain, vomiting, features of peritonitis, and pelvi-abdominal mass in contrast to vaginal bleeding/spotting/discharge seen in premenarchal girls. The most unique complication, in this case, was rupture into the peritoneal cavity resulting in significant hemoperitoneum causing an acute abdomen. Rupture of infantile uterine botryoid sarcoma and hemoperitoneum was reported earlier once in the Italian language, but details are not available.
Uterine BRMS with DICER1 mutation was found associated with metachronous cystic nephroma, as mentioned above. Two of 14 patients with cervical BRMS had pleuropulmonary blastoma and another had Sertoli–Leydig cell tumor of the ovary and nodular hyperplasia of the thyroid also. One of them with cervical BRMS and pleuropulmonary blastoma had DICER1 mutation of the BRMS. DICER1 mutation may be responsible for associated extrauterine tumors. The presence of BRMS of the cervix in two sisters in a family highlights the possible role of genetic factors in its development. According to the International classification system for childhood RMSs, Botryoid sarcoma is a superior prognosis variety. However, ERMS of the uterine corpus is high-grade malignant tumor probably from certain genetic mutations. DICER1 testing is of value in distinguishing between uterine ERMS and adenosarcoma; when DICER1 mutation is absent, it excludes ERMS. Malignant mixed Mullerian tumor or carcinosarcoma can grow in an exophytic manner from the uterine wall or cervix and have sarcomatous appearance grossly and microscopically; but unlike ERMS, malignant mixed Mullerian tumor typically occurs in older patients.
The ideal treatment of our case is neoadjuvant chemotherapy. Second look surgery (radical hysterectomy + proximal vaginectomy) is required only for gross residual disease who have failed to achieve a complete radiographic response within 6 months of induction chemotherapy and radiotherapy or those who have early progression. Ovarian transposition (oophoropexy) before radiation therapy should be considered.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]