Early-stage serous fallopian tube carcinoma

Abstract

Primary fallopian tube carcinoma (PFTC) is a rare disease. Its location, close association with epithelial ovarian carcinoma, and lack of specific signs and symptoms make diagnosis challenging especially in its early stages. We report a postmenopausal patient who presented with a 2-month history of abdominopelvic pain with watery vaginal discharge. Imaging findings showed a 7 cm complex left adnexal mass. The patient underwent a robotic-assisted total laparoscopic hysterectomy and bilateral salpingo-oophorectomy and surgical staging. Findings were significant for stage IA serous fallopian tube carcinoma. PFTC is sometimes missed preoperatively and intraoperatively. Available literature review has focused on the clinical and imaging characteristics of PFTC to aid in timely disease diagnosis. Minimally invasive surgery is a viable option in the diagnosis and management of early-stage ovarian cancer due to improved visualisation of pelvic structures, decreased length of hospital stay, decreased estimated blood loss and lower postoperative complication rates compared with laparotomy.

Background

Primary fallopian tube carcinoma (PFTC) is a rare disease, affecting 0.36 to 0.41 per 100 000 women per year. It falls within the category of epithelial ovarian cancer (EOC), which also incorporates ovarian and primary peritoneal carcinomas. Though rare, these cancers make up the fifth-leading cause of cancer-related deaths in the USA.¹ Early-stage PFTC is challenging to diagnose preoperatively, as patients tend to be symptom-free.² If the disease is caught early, the 5-year survival rate can be as high as 92%.¹ In addition, minimally invasive surgery has shown interesting potential for the management of early-stage epithelial ovarian cancer (eEOC). Compared with laparotomy, laparoscopy promises several advantages including improved patient outcomes. We present a rare case of serous fallopian tube carcinoma diagnosed in its early stages and managed robotically.

Case presentation

Our patient is a postmenopausal para 2 (P2) who presented with a 2-month history of pink, watery vaginal discharge and left-sided lower abdominal and pelvic pain. Physical exam findings were unremarkable and a review of systems was unremarkable. Her medical history was significant for type 2 diabetes mellitus, hyperlipidemia, hypertension and hypothyroidism. She denied any history of familial cancers. She was up-to-date with her health maintenance including a negative Pap smear in the year of diagnosis.

Investigations

Preoperative workup included a pelvic ultrasound, which showed a 7 cm complex multinodular predominantly solid mass in the left adnexa. Subsequently, a pelvic MRI captured a tubular mass immediately posterior to the left ovary, containing both cystic and solid components and measuring approximately 9 cm (figure 1). CT scan of the abdomen and pelvis confirmed previously obtained MRI findings and ruled out distant metastases. CA-125 was 19 U/mL.

Figure 1.

Axial T1 three-dimensional postcontrast MRI of the pelvis.

Differential diagnosis

Based on the patient’s symptoms, several differential diagnoses can be considered. Left-sided abdominal/pelvic pain may be significant for ovarian cysts, pelvic malignancy, tubo-ovarian abscess or diverticulitis. However, considering the chronicity of the patient’s symptoms, lack of fever and postmenopausal status, it is more likely that the patient is at increased risk for pelvic malignancy. Imaging investigations support a mass-like structure, and whether it is ovarian or tubular in nature is best confirmed with surgical intervention.

Treatment

The patient underwent a robotic-assisted total laparoscopic hysterectomy and bilateral salpingo-oophorectomy and staging including omentectomy and pelvic and para-aortic lymph node dissection. The left fallopian tube, including the specimen of interest, was excised in its entirety without any spillage of contents and placed in a specimen retrieval pouch. It was delivered through the vaginal cuff and sent for intraoperative frozen section consultation. Intraoperative histopathology was significant for a 9 cm left fallopian tube carcinoma. Grossly, the specimen appeared as a dilated, tubular structure; its lumen was obstructed by a solid, well-defined, exophytic mass with a pale-tan, soft-to-friable surface. The mass appeared to arise from the proximal ampulla of the left fallopian tube. The fimbriae were not identified. The final pathology report showed a poorly differentiated carcinoma of the left fallopian tube with serous features, FIGO stage IA (figure 2). The tumour was arranged in a SET (solid, endometrial-like, transitional) pattern with high-grade cytologic atypia and increased mitotic figures (figure 3). The tumour cells were positive for AE1/3, CK7, WT1, PAX8, ER (weak) and p16 (patchy) and negative for CK20, TTF-1, CDX2 and GATA3. Immunostain for p53 was equivocal due to suboptimal weak internal control. Pelvic washings demonstrated rare groups of atypical glandular cells admixed with mesothelial cells.

Figure 2.

Low-power view of tumour within the fallopian tube.

Figure 3.

High-power view of the tumour cells with enlarged nuclei, prominent nucleoli, nuclear pleomorphism and increased mitosis.

Outcome and follow-up

The patient’s postoperative course was uncomplicated. Multidisciplinary Cancer Conference recommendations included adjuvant chemotherapy (taxane and platinum doublet) as well as genetic testing, which revealed a PTEN deletion. The patient had undergone 4 months of chemotherapy. After completion of treatment, repeat CT scan of the abdomen and pelvis revealed no evidence of disease. The patient was advised to continue routine follow-up every 3 months with her gynaecological oncologist for fallopian tube cancer and CA-125 surveillance with repeat imaging recommended at 6, 12 and 24 months.

Discussion

Fallopian tube carcinoma most commonly arises as a result of a single-cell TP53 mutant epithelial layer that then develops into a serous tubular intraepithelial carcinoma (STIC). STIC lesions are characterised by atypical secretory fallopian tube epithelium with high proliferation rates and accumulation of aberrant p53 protein.¹ Alternatively, STIC lesions may develop from deletion or reduced expression of the tumour suppressor gene, PTEN. PTEN is lost in 33% of these lesions.³ STIC lesions have the potential to invade tubal stroma locally, developing into primary fallopian tube carcinoma, or spread onto the ovarian stroma or the peritoneum, developing into primary ovarian or peritoneal carcinoma.¹ Risk factors for epithelial ovarian carcinoma, and thus fallopian tube carcinoma, are those that increase lifetime ovulation cycles, such as nulliparity, early menarche and late menopause.¹ The most common histological subtype of PFTC is serous fallopian tube carcinoma.^{2 4}

Primary fallopian tube carcinoma is difficult to diagnose because symptoms and imaging findings are nonspecific. In contrast to ovarian cancer, PFTC is not routinely suspected in patients presenting with a complex pelvic mass. A study has shown that only about 4% of patients were diagnosed with PFTC preoperatively, 68.7% of patients were diagnosed at stage III/IV postoperatively and up to 50% of the disease was missed intraoperatively.² Another study determined that 10.5% of patients were diagnosed with PFTC preoperatively, 80.7% were misdiagnosed with ovarian cancer and 8.8% were misdiagnosed with endometrial cancer.⁴

Because of its misdiagnosis and rarity, special attention has been paid to understand the disease’s presentation pattern and characteristics on various imaging modalities. Historically, the clinical presentation of fallopian tube carcinoma has been described by Latzko’s triad, which includes intermittent profuse serosanguinous vaginal bleeding, colicky pain relieved by discharge and an abdominal or pelvic mass.² Latzko’s triad manifests in early-stage disease.⁴ However, the percentage of patients that actually present with all of these symptoms is small and was only reported in 7%–15% of patients.^{2 4} Other, atypical, symptoms of PFTC that have been documented include irregular menstruation, urinary frequency and urgency, generalised body aches, fever and lower extremity oedema.⁴

Imaging modalities such as ultrasound, CT scan and MRI have been used to aid in PFTC diagnosis. Ultrasound characteristics of PFTC may demonstrate a sausage-shaped or multilobular mass as well as low-impedance vascular flow within the solid components of the tumour.² PFTC on MRI presents as a small, sausage-like, solid unilateral mass with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images.⁵ However, in one study, preoperative MRIs accurately diagnosed only 43% of patients with PFTC.⁵ Findings of tubal carcinoma on imaging are similar to those of other pelvic morbidities such as tubo-ovarian abscess and ovarian tumours due to their close proximity.² Research has found that compared with epithelial ovarian cancer, PFTC on imaging presents smaller in size with a more homogenous signal in addition to hydrosalpinx and intrauterine fluid accumulation.⁵

Treatment of PFTC is dependent on the stage of disease. In early-stage disease, it is essential to undergo complete staging surgery, as up to 30% of patients who undergo staging surgery are ultimately upstaged, and surgical staging is an independent prognostic factor for improved disease-free and overall survival.^{1 2 6}

The role of minimally invasive surgery for staging of ovarian and other pelvic organ malignancies has been of recent interest. Laparoscopy has been shown to have several advantages over laparotomy for tumour debulking and staging including magnification of abdominal and pelvic anatomy, improved visualisation of pelvic structures, decreased length of hospital stay and decreased estimated blood loss.⁷ A systematic review investigating the efficacy and safety of minimally invasive surgery for treatment and staging of eEOC demonstrated similar findings as well as lower postoperative complication rates and shorter time to chemotherapy compared with open surgery. Upstaging and risk of cyst rupture were comparable among laparoscopic and laparotomic procedures.⁸ A retrospective cohort study found that the 5-year progression-free survival and overall survival rates of patients with eEOC who had undergone minimally invasive surgical staging were 84.0% and 92.5%, respectively.⁹

Minimally invasive surgery has demonstrated great promise in the field of gynaecological oncology. However, it must be carefully considered for appropriate candidates. Gallotta et al demonstrated that of those patients with eEOC who have undergone minimally invasive surgery, patients who had grade 3 disease had a negative prognostic value.⁹ Favourable characteristics that influenced progression-free survival include younger age, non-serous tumour histotype, tumour grades 1–2 and FIGO stage IA/IB.⁹ These findings should be kept in mind for surgical planning. The authors do not recommend debulking metastatic ovarian and fallopian tube cancers in a minimally invasive fashion. It is our practice to perform open primary cytoreductive surgery via laparotomy. With this case study, we aim to suggest the potential of a minimally invasive approach for patients least likely to experience a negative oncological outcome.

Our case study is of interest, as our patient was symptomatic and diagnosed with serous fallopian tube carcinoma at stage IA. Literature review has demonstrated that patients with early-stage disease may present with symptoms of Latzko’s triad. Our patient convincingly presented with all three criteria including pink, watery vaginal discharge, abdominal/pelvic pain and a pelvic mass on imaging. Imaging findings were quite the opposite of equivocal and were convincing of a mass of fallopian tube origin. Additionally, our patient had undergone minimally invasive surgery as part of the management of her early-stage disease, an intervention that is improving surgical efficiency and patient outcomes. Future studies and medical practice should continue to recognise and raise suspicion for early signs and symptoms, risk factors and imaging characteristics of PFTC to reduce morbidity and mortality from late-stage disease.

Learning points.

• Primary fallopian tube carcinoma, especially in its early stages, is sometimes missed preoperatively and intraoperatively since it is a rare disease.

• Patients with early-stage disease may present with symptoms of Latzko’s triad, including intermittent profuse serosanguinous vaginal bleeding, colicky pain relieved by discharge and an abdominal or pelvic mass.

• Minimally invasive surgery is a viable option in the diagnosis, staging and treatment of early-stage ovarian cancer due to improved visualisation of pelvic structures, decreased length of hospital stay, decreased estimated blood loss and lower postoperative complication rates compared with laparotomy.

Ethics statements

Patient consent for publication

Consent obtained directly from patient(s).