Neovaginal cancer after sigmoid vaginoplasty: Implications for postoperative cancer surveillance

Abstract

Background

Intestinal vaginoplasty can be performed as vaginal reconstruction procedure. Aa pedicled intestinal segment is isolated and transferred to the (neo)vaginal cavity to form the neovaginal lining.

Case

A 38-year old patient with Fanconi anemia and congenital vaginal absence, who underwent sigmoid vaginoplasty at the age of one, visited the outpatient clinic with a palpable neovaginal mass. At MRI examination, a neovaginal tumor was observed of approximately 25 × 10 mm without lymphadenopathy, which turned out to be a mucinous adenocarcinoma (T3N0M0). She underwent excision of the total mesocolon, including the sigmoid neovagina, coloanal anastomosis and anus.

Summary and Conclusion

Postoperative cancer surveillance seems justified after sigmoid vaginoplasty, even more so in patients with a (genetically) high risk of developing cancer and/or with a history of malignancy.

Introduction

Vaginoplasty, the surgical creation of a vagina, is indicated in:

• (1)women with congenital vaginal absence, such as is the case in various disorders of sexual development,
• (2)women with postablative vaginal absence and
• (3)transgender women, as primary or as revision vaginoplasty procedure.

For these purposes, different surgical techniques exist. Types of graft used for the vaginoplasty procedure are partial or full thickness skin grafts, pedicled groin flaps, pedicled intestinal segments, peritoneum and custom made molds. Each technique advantages and disadvantages.

In intestinal vaginoplasty, a pedicled intestinal segment is isolated and transferred to the (neo)vaginal cavity to form the neovaginal lining.¹ In literature, sigmoid or ileal segments are predominantly used. Subsequently, intestinal continuity is restored. Sigmoid vaginoplasty is performed laparotomically, laparoscopy-assisted, totally laparoscopic or robot-assisted.^2,3 After surgery, patients are able to engage in penetrative sexual intercourse and generally report good functional and sexual outcomes.4, 5, 6, 7 Advantages of this technique comprise natural lubrication, no postoperative shrinkage of tissue and sufficient tissue to achieve adequate neovaginal depth.¹ Disadvantages of this technique comprise the need for intra-abdominal intestinal surgery and the risk of postoperative bowel complications, such as diversion neovaginitis, neovaginal inflammatory bowel diseases and malignancies. The incidence of malignancy of the sigmoid neovagina is unknown and this has only been described in case reports. In this case report, a 38-year old woman with Fanconi anemia is described with neovaginal cancer after sigmoid vaginoplasty at the age of 1.

Case

A 38-year old woman visited our outpatient clinic with a palpable neovaginal mass. Her medical history comprised Fanconi anemia with an atrial septal defect, radial and thumb hypoplasia, renal agenesis, and congenital absence of the vagina, uterus and anus. Fanconi anemia was diagnosed in her childhood using the mitomycin C test. At an early age, she underwent a colostomy procedure.

To correct the anal and vaginal atresia, she underwent a laparotomic pull through procedure at the age of 1. A neovagina was formed from the distal rectosigmoid segment, which was flipped over and transferred to the neovaginal cavity, much like a J-pouch. The neovaginal top was stapled. The perineum was incised and a neo-introital and colo-anal anastomosis were performed.

At current physical examination, a mass of approximately 2 cm in diameter was palpated. In a biopsy specimen, poorly-differentiated adocarcinoma of the sigmoid neovagina was found. At MRI examination, a tumor was observed of approximately 25 × 10 mm without lymphadenopathy (Figure 1). No distant metastases were seen at thoraco-abdominal CT examination. At multidisciplinary consultation, resection of the neovagina was proposed. The patient expressed the wish for immediate neovaginal reconstruction during the same procedure.

Figure 1.

MRI examination of the sigmoid neovaginal malignancy. Sagittal (upper left and right) and transverse (lower left and right) planes.

At surgery, the initial laparoscopic approach was converted to laparotomy because of extensive intra-abdominal adhesions. At intra-abdominal examination, the tumor was palpable at the neovaginal top. The neovagina shared the mesocolon with part of the descending colon, neorectum and -anus.

To achieve an adequate oncological excision, the total mesocolon had to be excised, including the sigmoid neovagina, coloanal anastomosis and anus. A frozen section was performed, in which radical tumor excision was observed. The left-sided colon was mobilized. The descending colon was brought down and a J-pouch construction was formed. A direct neovaginal reconstruction was performed by separating the J-segment and using it to form the new neovaginal cavity and neo-anus. A stapled coloanal anastomosis was performed. There were no intra- or postoperative complications. The hIstopathology of the resection specimen reported presence of a mucinous adenocarcinoma, with malignant cells reaching into the muscular and fatty tissue. The tumor was radically excised. There were no lymph node metastases (T3N0M0).

Summary and conclusion

In this report, a 38-year old woman with Fanconi anemia with neovaginal cancer of the sigmoid neovagina is presented. Treatment consisted of surgical removal of the neovagina, -rectum and –anus. Direct neovaginal reconstruction was successfully performed using colonic tissue. Cancer of the (intestinal) neovagina has only been reported previously in singular case studies.⁸ An overview of literature on malignancies of the intestinal neovagina is provided in Supplementary Table 1. In these case reports, describing a total of fourteen patients, the median time between sigmoid vaginoplasty and cancer diagnosis is 22 years (range 3–48).

The sigmoid can be used for surgical neovaginal reconstruction. Macroscopic and microscopic inflammatory changes of the sigmoid after its use in sigmoid vaginoplasty have been observed in the majority of patients.⁹ Lack of luminal nutrients due to fecal stream diversion is most likely the cause of these changes. Chronic inflammation, such as in ulcerative colitis, is considered a major risk factor for colorectal malignancy formation. Because of this we put forward, that patients who underwent sigmoid vaginoplasty are presumably at high(er) risk of developing a malignancy in this intestinal segment. Co-infection with human papilloma virus may be an additional risk factor, such as in rectal carcinoma in men-having-sex-with-men. HPV-related carcinomas have been reported in skin-lined neovaginas.¹⁰

Little is known on the incidence of neovaginal malignancies, because this type of surgery is not frequently performed and there is no clear consensus on postoperative follow-up in these patients. Moreover, surveillance strategies for colorectal cancer are usually not applied in individuals with a neovagina (at least not for the neovaginal part).

Sigmoid vaginoplasty is performed in non-transgender women with congenital or postablative vaginal absence and transgender women as surgical vaginal reconstruction. We advise careful patient selection before performing this procedure. For example, patients with a history of ulcerative colitis are not ideal candidates for this procedure and may be at high-risk for developing inflammation-induced neovaginal cancer. To date, no clear guidelines exist on the postoperative follow-up of these patients. To provide adequate oncological follow-up, we propose the following:

• -Surveillance of a sigmoid neovagina seems recommendable 10–20 years after vaginal (re)construction with follow-up in 5 – year intervals, or more frequent in case of dysplasia, similarly to patients with ulcerative colitis.
• -In patients with a genetic predisposition to colorectal malignancies, sigmoid vaginoplasty may not be the first choice as (neo)vaginal reconstruction technique.
• -Postoperative sigmoid neovaginal cancer surveillance seems just, especially in those patients with a genetically high risk of cancer development and/or with a history of malignancy.
• -If in these patients with a sigmoid vagina, cancer of the native intestinal tract is diagnosed, examination of the sigmoid neovagina is mandatory, and the other way around in case of neovaginal cancer.
• -When treating sigmoid neovaginal cancer patients, a multidisciplinary approach with gastroenterologists, gastro-intestinal/oncological surgeons and reconstructive surgeons seems preferable.

Ethical aprovement statement

The study manuscript was approved by our institutional medical ethical committee (METC).

Other statement

The authors adhered to the STROBE guidelines.

Declaration of competing interest

None to declare for all authors.