A Rare Case of Bilateral Primary Fallopian Tubal Carcinoma
J Cancer Sci Oncol
Article Type : Case Reports
Lara Strakian1, Jing Song1 and Murad Al-Aker2
1Obstetric and Gynecology Consultant, Department of Obstetrics & Gynecology, Campbelltown Hospital NSW, Australia
2Consultant Gynecological Oncologist, Liverpool Cancer Therapy Centre and Campbelltown Hospital NSW, Australia
A middle age nulliparous woman presented with vaginal bleeding and pelvic pain on a background of positive family history for ovarian cancer. Underwent hysterectomy and bilateral salpingectomy. Histopathology confirms serous tubal intra epithelial carcinoma of bilateral fallopian tubes with benign uterus and cervix. Despite the positive family history, the BRCA gene screen was negative. Subsequently, she had a surgical staging with bilateral oophorectomy and received six courses of chemotherapy. She is currently on regular follow up with gynaecological oncology team.
Keywords: Fallopian; Bilateral; Tubes; Carcinoma; Cancer; Ovarian; Pelvic
Primary fallopian tubal carcinoma (PFTC) is a rare form of gynaecological malignancy. Nulliparous women with prolonged life time of menstruation and positive family history of ovarian cancer are at high risk of PFTC. According to the literatures there is strong association between fallopian cancer with ovarian and peritoneal cancer and also the BRCA gene mutation. The presenting features of PFTC are nonspecific in form of abdominal bloating, distension and rarely watery vaginal discharge. Hence, the preoperative diagnosis of PFTC is difficult. The operative staging is the main way to guide the treatment. Treatment includes Hysterectomy, bilateral oophorectomy, Lymphadenectomy with or without chemotherapy.
45 years old nulliparous woman presented to the emergency department of Campbelltown Hospital with a history of heavy vaginal bleeding and abdominal pain for one day duration. Her menstrual history was unremarkable until 5 months prior to her presentation to the emergency when she started having irregular and heavy menstrual periods. Her past medical history include morbid obesity which she had gastric sleeve and subsequently had significant weight loss from 128 kg to 65 kg. She has had hiatus hernia and bilateral breast lumpectomy for benign lesions. Her family history is significant for ovarian cancer with her mother having ovarian cancer in her 50s and paternal aunt was diagnosed with ovarian cancer in her 50s as well. Her maternal aunt had multiple myeloma in her 50s and paternal grandmother had bowel cancer. In terms of her gynaecological history, she had regular normal pap smears and regular menstrual monthly period of 5 days duration with average blood loss. On assessment, she was not pale and abdomen was soft with mild right iliac fossa tenderness on deep palpation. Bimanual pelvic examination revealed average size uterus, no cervical motion tenderness and no adnexal masses but mild right adnexal tenderness. Speculum examination did not reveal any abnormality either. Blood tests for BHCG was negative, haemoglobin level and inflammatory markers were within normal range. Pelvic ultrasound showed pedunculated fibroid of the posterior uterine wall of 39 x 21 x 36 mm, hyper echogenic endometrium of 10.2 mm thickness and left ovarian bilocular benign looking cyst. Conservative options of management include endometrial sampling with IUCD bleeding was discussed with the patient however she opted for hysterectomy as was not planning for pregnancy in the future. She underwent laparoscopic hysterectomy along with bilateral salpingectomy in view of her high risk history for ovarian cancer. Intraoperatively, both ovaries and tubes looked grossly normal. The histopathology was suggestive of high grade serous tubal carcinoma of bilateral fallopian tubes, benign uterus and cervix with 35 mm posterior cervical fibroid. Ovarian tumour markers were negative. Chest x-ray and abdominal pelvic CT scan did not reveal any lymph nodes or metastases. Subsequently, she underwent laparoscopic bilateral oophorectomy with surgical staging (peritoneal washing, infra colic omentectomy, appendectomy and right pelvic lymph node dissection). The histopathology revealed incidental small focus of atypical glands in the right ovary which was not in keeping with serous carcinoma and eight negative pelvic lymph nodes with otherwise no evidence of metastases elsewhere. Following to this, she had adjuvant chemotherapy with Carboplatin and paclitaxel every 3 weeks. She recovered well with no complications.
Primary fallopian tubal carcinoma (PFTC) is one of the rarest gynaecological malignancies. The percentage of PFTC among all genital malignancies is 0.14-1.8% [1]. The total number of PFTC cases that have been reported so far in the literature is 1200 [2,3]. The average incidence of PFTC in United State is 3·6 for every million women in a year [4]. Whereas in England & Wales, the number of annually registered PFTC cases is 40 cases a year [5]. In literatures, fallopian carcinoma is always grouped with ovarian and primary peritoneal carcinoma under the broad name ‘Epithelial ovarian cancer’ EOC. The pathophysiology of PFTC is unknown. Certain factors such as increased parity, use of contraception and the longer duration of breast feeding are postulated to be protective against EOC by reducing the ovulation time during the reproductive age [6-8]. Reversely, nulliparity, early menarche and late menopause are thought to increase the risk of EOC. There is fivefold higher incidence of bilateral fallopian tube carcinoma in infertile women [9], with a significant association between fallopian tube carcinoma and BRCA mutation gene [10-12]. The fallopian tubes were the main primary site of origin of malignancies among women with BRCA positive gene who underwent a risk reduction surgery [10]. Additionally, the risk of serous cancer in the context of BRCA mutation gene are attributed to the fimbrial part of the fallopian tube rather than the ovary. PFTC is common between 40 and 60 years of age [13] with the peak age of incidence being 55 years of age [14]. The clinical manifestations of PFTC are nonspecific between abdominal pain and or vaginal bleeding. However, the syndrome of ‘Hydrops tubae profuse’ which is the classic triad of lower abdominal colic with serosanguinous profuse vaginal discharge & abdominopelvic mass, has been reported in only 15% of cases [15]. Rarely, PFTC can present as tubo ovarian abscess [14]. According to the literature, fallopian carcinoma can rarely be diagnosed pre-operatively as most of the time it is misdiagnosed as ovarian carcinoma. A high serum CA 125 in suspected cases of fallopian tube carcinoma can aid in the diagnosis as CA 125 antigen is produced by fallopian tube carcinoma [14]. CA 125 level are elevated in 65% of PFTC cases [16,17]. Elevated serum CA 125 was found to be associated with advanced or recurrent fallopian malignancy [15,18]. Additionally, studies have agreed that post-operative CA125, is a valuable marker for disease progression and follow up [19,20]. Imaging as in any other gynaecological malignancies should be part of the work up for fallopian carcinoma. Sonographic findings of fallopian cancer are nonspecific in form of adnexal mass which can mimic other conditions such as tubo ovarian abscess, ectopic pregnancy or ovarian carcinoma. However, fallopian carcinoma can be seen as a sausage shape, multilobulated or cogwheel like mass on ultrasound. Transvaginal ultrasound with colour Doppler was found to be more useful in the diagnosis of fallopian carcinoma by detecting neovascularization of the fallopian tubes [21]. Moreover, three-dimensional Doppler ultrasound can demonstrate irregularities of the fallopian wall which could aid in the pre-operative diagnosis of fallopian tube carcinoma [22]. Recent publications have demonstrated the role of cervical pap smear in preoperative diagnosis of fallopian tube carcinoma. Pectasides D et al noted that abnormal pap smear with unremarkable colposcopy, endometrial curettage and cervical biopsy should raise the suspicion of PFTC [23]. Cases of fallopian tube carcinoma have been reported in women with whom the sole finding was the presence of malignant cells and psammoma bodies in the cervical smear [24-27]. The different histological types of PFTC are: serous, endometriod, mixed, undifferentiated, clear cell, transitional and mucinous with the serous being the most common type followed by the endometrioid [23]. Staging of PFTC is similar to the surgical staging of ovarian carcinoma depending on the intraoperative findings of the extent of the metastases of the tumour. PFTC behave like primary ovarian cancer in terms of the pattern of the spread. However, unlike primary ovarian cancer, PFTC can often be detected at earlier stages. Lymphatic spread to the peritoneal cavity has been detected in about 80% of advance PFTC cases [28]. Surgical staging of the tumour is the guide for post-operative treatment. The surgery includes total abdominal hysterectomy, bilateral salpingo-oophorectomy along with omentectomy and lymphadenectomy with the latter being an essential component of the surgery.
Although PFTC is one of the rarest malignancies, it can have detrimental impact on the quality and quantity of the woman’s life and her family. Hence, bilateral salpingectomy is highly recommended for those women who are at high risk for ovarian cancer. Given the rarity and the nonspecific presentation of PFTC, efforts is to be focused on more literature studies to better inform the gynaecological practitioner about this type of malignancy and to aid in identifying possible ways for early recognition and diagnosis and thereby a better prognosis and outcome.
Patient consent was obtained.
Corresponding Author: Lara Strakian, Lecturer, Department of Obstetrics & Gynecology, Campbelltown Hospital, Australia Copyright: © 2023 All copyrights are reserved by Lara Strakian, published by Coalesce Research Group. This work is licensed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.
