AUTHOR AND EDITOR INFORMATION

Section 1 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Background

In 1847, Renaud first described fallopian tube malignancy. In 1888, Orthmann submitted the first genuine case report.

The broad ligament is a double fold of peritoneum, which is formed by the reflection of the peritoneum off the pelvic floor and the lateral pelvic wall. Most tumors are benign cysts, but malignant tumors are categorized as either primary or secondary.

Primary malignancies of the broad ligament include those of müllerian origin (ie, serous carcinoma [CA], papillary CA, cystadenocarcinoma, endometrioid CA, clear cell CA), urothelium transitional cell CA, mesenchymal sarcoma or histiocytoma, and pheochromocytoma.

Secondary malignancies of the broad ligament include metastatic cancers from endometrial, cervical, and ovarian CA.

A primary malignancy is diagnosed based on its location within or on the surface of the broad ligament and by virtue of the complete separation of the tumor from the uterus and ovaries.

Pathophysiology

Fallopian tube malignancy usually starts as a dysplasia or CA in situ. Typically, transition to adenocarcinoma is observed.

The etiology of malignancies of the broad ligament is unknown, although they are associated with endometriosis.

Frequency

United States

Fallopian tube CA comprises 1% of all gynecologic cancers. The average annual incidence is 3.6 cases per million women.

Mortality/Morbidity

On average, the 5-year survival rate is 51%; the rate for stage I disease is 65%, stage II disease is 50-60%, and stage III and stage IV disease is 10-20%.

Age

Incidence increases with age but peaks at 60-66 years.

CLINICAL

Section 3 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

History

• Malignant lesions of the fallopian tube
• • Patients may present with pelvic pain, a pelvic mass, postmenopausal bleeding, and serosanguineous vaginal discharge.
  • The classic description of hydrops tubae profluens, which is characterized by colicky lower abdominal pain relieved by a profuse, serous, watery, yellow, intermittent, vaginal discharge, usually is not found.
• Malignant lesions of the broad ligament
• • A clinical history of vague abdominal pain may be present. Upon examination or with abdominal exploration, an adnexal mass is found.
  • Rarely, it can manifest as an acute abdominal emergency, simulating appendicitis.

Physical

• Physical examination findings are not specific; a pelvic mass usually is present, with or without ascites.
• Diagnostic criteria include the following:
• • Grossly, the main tumor should be in the fallopian tube.
  • Histologically, the tubal mucosa should be involved, with a papillary pattern.
  • The tubal wall, if involved, and a transition from benign to malignant tubal epithelium should be identified.
  • The lesion is a more advanced stage of tubal tumor than the other tumors.

Causes

• The exact etiology is unknown.
• • Infertility and chronic salpingitis were believed to lead to an increase in incidence, but this theory has not been proven. However, malignancy has been associated with tuberculous salpingitis.
  • Similar to ovarian malignancy, a BRCA germline mutation and TP53 mutation are associated with fallopian tube malignancy.

DIFFERENTIALS

Section 4 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Benign fallopian tube disease salpingitis (eg, tuberculosis salpingitis)
Benign ovarian disease
Ovarian tumor of low malignant potential
Malignant epithelial ovarian tumors
Broad ligament tumors

WORKUP

Section 5 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Lab Studies

• Investigations usually are for postmenopausal bleeding and include the following:
• • Papanicolaou smear test, showing malignant glandular cells
  • Endometrial biopsy
• The cancer antigen 125 (CA-125) tumor marker assay is performed for follow-up study as a marker of response to therapy.

Imaging Studies

• Hysterosalpingogram can help detect intraluminal growth.
• Color Doppler can help detect arteriovenous shunts, microaneurysms, tumoral lakes, vascular blind ends, dichotomous branching, neovascularization, and low resistance indices.
• Three-dimensional ultrasound can help detect papillary protrusions, pseudoseptae, tumoral lakes, microaneurysms, and arteriovenous shunting.
• Ultrasound helps detect an adnexal mass, especially a solid mass corresponding with the expected location of the fallopian tubes in association with normal ovaries or as a sausage-shaped cystic mass with papillary projections.

Histologic Findings

Type of CAs found include serous CA (50%), endometrioid CA (25%), transitional cell CA (11.7%), undifferentiated CA (7.8%), mixed CA (3.9%), and clear cell CA (1.9%).

Immunohistochemistry: Glucose transporter (GLUT1) immunostaining of fallopian tube adenocarcinoma was stronger and more extensive than staining of benign tubal epithelium. GLUT1 positivity is observed in regions most distal from stromal capillaries, suggesting hypoxia-driven GLUT1 induction. On average, GLUT1 staining in primary fallopian tube cancer was less extensive than in primary ovarian adenocarcinomas (Kalir, 2005).

Staging

Although the fallopian tubes are derived from the same embryonic structure as the uterus, histologically and clinically, malignant lesions of the fallopian tubes behave like ovarian tumors. Unlike ovarian tumors, 50% of fallopian tube tumors are stage I and II, whereas more than 50% of ovarian malignancies are usually in stage III and IV. Fallopian tube carcinomas have a predilection for metastasis to retroperitoneal lymph nodes in contrast to intraperitoneal spread of ovarian carcinomas.

• International Federation of Gynecology and Obstetrics (FIGO) fallopian tube cancer staging is as follows:
• • Stage 0 - CA in situ (limited to tubal mucosa)
  • Stage I - Growth limited to the fallopian tubes
    • Stage Ia - Growth limited to 1 tube, with extension into the submucosa and/or muscularis but not penetrating the serosal surface; no ascites
    • Stage Ib - Growth limited to both tubes with extension into the submucosa and/or muscularis but not penetrating the serosal surface; no ascites
    • Stage Ic - Tumor stage 1a or 1b but with tumor extension through or onto the tubal serosa, or with ascites present containing malignant cells, or with positive peritoneal washings
  • Stage II - Growth involving 1 or both fallopian tubes with pelvic extension
    • Stage IIa - Extension and/or metastasis to the uterus and/or ovaries
    • Stage IIb - Extension to other pelvic tissues
    • Stage IIc - Tumor stage IIa or IIb, with ascites present containing malignant cells, or with positive peritoneal washings
  • Stage III - Tumor involving 1 or both fallopian tubes, with peritoneal implants outside the pelvis and/or positive retroperitoneal or inguinal nodes; superficial liver metastasis equals stage III; tumor appears limited to true pelvis but has histologically proven malignant extension to the small bowel or omentum
    • Stage IIIa - Tumor grossly limited to the true pelvis, with negative nodes but with histologically confirmed microscopic seeding or abdominal peritoneal surfaces
    • Stage IIIb - Tumor involving 1 or both fallopian tubes, with histologically confirmed implants on abdominal peritoneal surfaces, none exceeding 2 cm in diameter; lymph node findings negative
    • Stage IIIc - Abdominal implants larger than 2 cm in diameter and/or positive retroperitoneal or inguinal nodes
  • Stage IV - Growth involving 1 or both fallopian tubes, with distant metastases; if pleural effusion is present, positive cytology results necessary to be stage IV; parenchymal liver metastases equal stage IV
• Staging for fallopian tube CA is via the surgical pathological system. Operative findings designating stage are determined before tumor debulking.
• Modified FIGO staging for fallopian tube CA is as follows:
• • Stage 0 - CA in situ (limited to tubal epithelium)
  • Stage I - Growth limited to tube
    • Stage IA - Growth limited to 1 fallopian tube, without extension through or onto serosa, ascites containing malignant cells, or positive peritoneal washings
    • Stage IA to 0b - Growth limited to 1 fallopian tube, with no extension into lamina propria
    • Stage IA to 1b - Growth limited to 1 fallopian tube, with extension into lamina propria but no extension into muscularis
    • Stage IA to 2b - Growth limited to 1 fallopian tube, with extension into muscularis
    • Stage IB - Growth limited to both fallopian tubes, without extension through or onto serosa, ascites containing malignant cells, or positive peritoneal washings
    • Stage IB to 0b - Growth limited to both fallopian tubes, with no extension into lamina propria
    • Stage IB to 1b - Growth limited to both fallopian tubes, with extension into lamina propria, but not extension into muscularis
    • Stage IB to 2b - Growth limited to both fallopian tubes, with extension into muscularis
    • Stage IC - Tumor either stage IA or IB, but with extension through or onto tubal serosa, with ascites containing malignant cells, or with positive peritoneal washings
    • Stage I(F) - Tumor limited to fimbriated end of fallopian tube(s), without invasion of tubal wall
  • Stage II - Tumor involving 1 or both fallopian tubes, with pelvic extension
    • Stage IIA - Extension and/or metastasis to uterus and/or ovaries
    • Stage IIB - Extension to other pelvic tissues
    • Stage IIC - Tumor either stage IIA or IIB, with ascites containing malignant cells or with positive peritoneal washings
  • Stage III - Tumor involving 1 or both fallopian tubes, with peritoneal implants outside pelvis, including superficial liver metastasis, and/or positive retroperitoneal or inguinal nodes; tumor limited to pelvis except for histologically proven extension to small bowel or omentum
    • Stage IIIA - Tumor grossly limited to pelvis, with negative nodes but with histologically confirmed microscopic seeding of abdominal peritoneal surfaces
    • Stage IIIB - Tumor involves 1 or both fallopian tubes, with grossly visible histologically confirmed implants of abdominal peritoneal surfaces, none exceeding 2 cm in diameter; lymph node findings negative
    • Stage IIIC - Abdominal implants larger than 2 cm in diameter and/or positive retroperitoneal or inguinal nodes
  • Stage IV - Growth involving 1 or both fallopian tubes, with distant metastases, including parenchymal liver metastases; if pleural effusion present, fluid must be positive cytologically for malignant cells
  • Modification in terminology - Modifications to accommodate subsets of tumor that otherwise cannot be assigned a stage or to distinguish among subsets that may differ in their associated prognosis

TREATMENT

Section 6 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Medical Care

Medical care depends on frozen section and pathology results, as follows:

• Stage 0 - CA in situ
  • Negative cytology results - No further treatment
  • Positive cytology results - Intraperitoneal phosphorous P 32 (³²P) or short-term cisplatin therapy
• Stage I
• • Negative cytology results - No treatment or short-term cisplatin therapy
  • Positive cytology results - Intraperitoneal ³²P or short-term cisplatin therapy
• Stage II-IV
  • Bulky residual disease or positive nodes - Cisplatin-containing regimens
  • No residual disease, negative nodes - Short-term cisplatin or intraperitoneal ³²P therapy
  • Residual disease in pelvis - Cisplatin regimen or whole-abdomen radiation with pelvic boost; however, as with ovarian malignancy, patients with fallopian tube malignancy also have an increase in the 5-year survival rate with Taxol and cisplatin therapy
• Various chemotherapy cycles are as follows:
• • Cyclophosphamide, doxorubicin, and cisplatin
  • Cyclophosphamide and cisplatin
  • Taxol and cisplatin
  • Topotecan alone - Used for first-line chemotherapy and can be used for recurrent disease
• Treatment of broad ligament malignancy is similar to that for fallopian tube malignancy except in young patients, in whom wide local excision can be adequate.

Surgical Care

Surgical care includes (1) total abdominal hysterectomy with bilateral salpingo-oophorectomy, (2) omentectomy and peritoneal washing, and (3) selective pelvic and para-aortic lymphadenectomy.

FOLLOW-UP

Section 7 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Further Outpatient Care

• Patients usually are evaluated using the CA-125 assay to monitor response to therapy. If a rise in CA-125 is noted, investigations such as CT scan and laparoscopy can be performed. Any evidence of disease can be treated with chemotherapy, debulking surgery, or both.

Prognosis

• Good prognostic factors are as follows:
• • Negative peritoneal cytology
  • No residual disease at primary cytoreductive surgery
  • Disease limited to the pelvis
  • Abnormal vaginal bleeding as a presenting symptom
  • Negative second look laparotomy
• Poor prognostic factors are as follows:
• • Advanced stage of the disease
  • Absence of fimbriated end closure in stage 1 disease
  • The presence of a TP53 mutation
• The presence or absence of invasion of tubal wall, the depth of invasion when present, and the location of the tumor within the tube (ie, fimbriated or nonfimbriated) are prognostic variables.
• The presence of ascites and the patient's age do not seem to affect prognosis because the prognosis depends on the location of the tumor within the fallopian tube and the depth of invasion of tumor; therefore, Navani suggests modification of the FIGO staging system.

MISCELLANEOUS

Section 8 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Fallopian tube and broad ligament malignancies are rare gynecological malignancies. Diagnosing these malignancies at an early stage is difficult because of the lack of symptoms or the presence of nonspecific symptoms. Diagnosis usually is an incidental finding; therefore, medicolegally, it is possible that patients can sue because of a failure to diagnose the cancer at an early stage or preoperatively. In addition, no standardized treatment exists. Usually, these conditions are treated in a manner similar to that used for ovarian cancer intraoperatively. Chemotherapy or radiotherapy after surgery depends on individual preferences.

REFERENCES

Section 9 of 9

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

• Alvarado-Cabrero I, Young RH, Vamvakas EC. Carcinoma of the fallopian tube: a clinicopathological study of 105 cases with observations on staging and prognostic factors. Gynecol Oncol. Mar 1999;72(3):367-79. [Medline].
• Aslani M, Scully RE. Primary carcinoma of the broad ligament. Report of four cases and review of the literature. Cancer. Oct 1 1989;64(7):1540-5. [Medline].
• Ben-Hur H, Dgani R, Ben-Arie A. Diagnostic dilemmas and current therapy of Fallopian tube cancer. Eur J Gynaecol Oncol. 1999;20(2):108-9. [Medline].
• Berek J, Hacker N, eds. Practical Gynecologic Oncology. 3rd ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2000:. 546.
• Danforth DN, Scott JR. Diseases of the Ovary and Fallopian Tubes. In: Danforth DN, Scott JR, Di Saia PJ, Hammond CB, Spellacy WN, eds. Danforth's Obstetrics & Gynecology. 8th ed. Philadelphia, Pa: Lippincott Raven; 1999:. 889.
• Dieste MC, Lynch GR, Gordon A. Malignant fibrous histiocytoma of the broad ligament: a case report and literature review. Gynecol Oncol. Oct 1987;28(2):225-9. [Medline].
• Disaia PJ, Creasman WT. Fallopian Tube Cancer. In: Creasman WT, Doherty M, Disaia PJ, Dinh TV, Hannigan EV, eds. Clinical Gynecologic Oncology. 5th ed. St. Louis, Mo: Mosby-Year Book; 1997:. 375-80.
• Dunton CJ, Neufeld J. Complete response to topotecan of recurrent fallopian tube carcinoma. Gynecol Oncol. Jan 2000;76(1):128-9. [Medline].
• Fedele L, Cittadini E, Bortolozzi G. Successful in vitro fertilization and embryo transfer after limited surgical treatment for tubal adenocarcinoma. Cancer. Oct 1 1989;64(7):1546-7. [Medline].
• Kalir T, Rahaman J, Hagopian G, et al. Immunohistochemical detection of glucose transporter GLUT1 in benign and malignant fallopian tube epithelia, with comparison to ovarian carcinomas. Arch Pathol Lab Med. May 2005;129(5):651-4.
• Kurjak A, Kupesic S, Jacobs I. Preoperative diagnosis of the primary fallopian tube carcinoma by three- dimensional static and power Doppler sonography. Ultrasound Obstet Gynecol. Mar 2000;15(3):246-51. [Medline].
• Latner AL, Turner GA. Effect of aprotinin on immunological resistance in tumour-bearing animals. Br J Cancer. May 1976;33(5):535-8. [Medline].
• Loverro G, Cormio G, Renzulli G. Serous papillary cystadenoma of borderline malignancy of the broad ligament. Eur J Obstet Gynecol Reprod Biol. Aug 1997;74(2):211-3. [Medline].
• Navani SS, Alvarado-Cabrero I, Young RH. Endometrioid carcinoma of the fallopian tube: a clinicopathologic analysis of 26 cases. Gynecol Oncol. Dec 1996;63(3):371-8. [Medline].
• Orthmann EG. Primareskarzinom in Einertuberkulosen. Ztschr Geburtsh Gynaek. 1888;15:212.
• Ricci JV. One Hundred Years of Gynecology. Philadelphia, Pa: Blackiston; 1945.
• Rose PG, Shrigley R, Wiesner GL. Germline BRCA2 mutation in a patient with fallopian tube carcinoma: a case report. Gynecol Oncol. May 2000;77(2):319-20. [Medline].
• Rosen AC, Ausch C, Hafner E. A 15-year overview of management and prognosis in primary fallopian tube carcinoma. Austrian Cooperative Study Group for Fallopian Tube Carcinoma. Eur J Cancer. Oct 1998;34(11):1725-9. [Medline].
• Rosen AC, Ausch C, Klein M. p53 expression in fallopian tube carcinomas. Cancer Lett. Aug 1 2000;156(1):1-7. [Medline].

Article Last Updated: Jun 12, 2006