doi:10.1111/jog.14522

J. Obstet. Gynaecol. Res. Vol. 46, No. 12: 2474–2487, December 2020

Clinical practice guidelines for the treatment of extragenital
endometriosis in Japan, 2018
Tetsuya Hirata1,2, Kaori Koga2, Kentaro Kai3, Hidetaka Katabuchi4, Mari Kitade5,
Jo Kitawaki6, Masatoshi Kurihara7, Naoko Takazawa8, Toshiaki Tanaka9,10,
Fuminori Taniguchi11, Jun Nakajima12, Hisashi Narahara3, Tasuku Harada11,
Shigeo Horie8, Ritsuo Honda4, Koji Murono9, Kotaro Yoshimura13 and Yutaka Osuga2
1

Department of Obstetrics and Gynecology, Doai Kinen Hospital, Tokyo, Japan
Department of Obstetrics and Gynecology, University of Tokyo, Tokyo, Japan3
Department of Obstetrics and Gynecology, Oita University, Oita, Japan
4
Department of Obstetrics and Gynecology, Kumamoto University, Kumamoto, Japan
5
Department of Obstetrics and Gynecology, Juntendo University, Tokyo, Japan
6
Department of Obstetrics and Gynecology, Kyoto Prefectural University, Kyoto, Japan
7
Pneumothorax Research Center and Division of Thoracic Surgery, Nissan Tamagawa Hospital, Tokyo, Japan
8
Department of Urology, Juntendo University, Tokyo, Japan
9
Department of Surgical Oncology, University of Tokyo, Tokyo, Japan10
Department of Surgery, International Catholic Hospital, Tokyo, Japan
11
Department of Obstetrics and Gynecology, Tottori University, Tottori, Japan
12
Department of Thoracic Surgery, University of Tokyo, Tokyo, Japan
13
Department of Plastic Surgery, Jichi Medical University, Shimotsuke, Japan
2

Abstract
The aim of this publication is to disseminate the clinical practice guidelines for the treatment of intestinal,
bladder/ureteral, thoracic and umbilical endometriosis, already published in Japanese, to non-Japanese
speakers. For developing the original Japanese guidelines, the clinical practice guideline committee wasformed by the research team for extragenital endometriosis, which is part of the research program of intractable disease of the Japanese Ministry of Health, Labor and Welfare. The clinical practice guideline committee formulated eight clinical questions for the treatment of extragenital endometriosis, which were intestinal,
bladder/ureteral, thoracic and umbilical endometriosis. The committee performed a systematic review of theliterature to provide responses to clinical questions and developed clinical guidelines for extragenital endometriosis, according to the process proposed by the Medical Information Network Distribution Service. The
recommendation level was determined using modiﬁed Delphi methods. The clinical practice guidelines wereofﬁcially approved by the Japan Society of Obstetrics and Gynecology and the Japan Society of Endometriosis. This English version was translated from the Japanese version.
Key words: bladder/ureteral endometriosis, guidelines, intestinal endometriosis, thoracic endometriosis,
umbilical endometriosis.

Received: August 17 2020.
Accepted: September 28 2020.
Correspondence: Dr Yutaka Osuga, Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo,
Bunkyo-ku, Tokyo, Japan. Email: yutakaos-tky@umin.ac.jp

2474© 2020 The Authors. Journal of Obstetrics and Gynaecology Research published by John Wiley & Sons Australia, Ltdon behalf of Japan Society of Obstetrics and Gynecology.
This is an open access article under the terms of the Creative Commons Attribution License, which permits use,
distribution and reproduction in any medium, provided the original work is properly cited.

Guideline for extragenital endometriosis

Introduction
Endometriosis is deﬁned as the presence ofendometrium-like tissue in organs other than theuterus. It has been reported that 5–10% of women of
reproductive age experience endometriosis.1,2 Endometriosis is an estrogen-dependent inﬂammatory disease that causes pelvic pain and infertility.
Endometriosis usually occurs in the ovaries, ligaments
and peritoneal surface, and less commonly in theintestine, bladder, ureter, abdominal wall, thoracic
cavity and other organs.3 Extragenital endometriosis
is a rare disease for which limited information isavailable in the literature. As extragenital endometriosis occurs in various organs other than gynecologicalorgans and presents with non-gynecological symptoms, multidisciplinary collaboration is important for
its optimal management and treatment. The clinicalpractice guidelines was published for the treatment ofintestinal, bladder/ureteral, thoracic and umbilicalendometriosis in Japan in 2018.4 The objective of theguidelines was to enable users to make their practiceevidence-based and to minimize any variations inclinical practice for extragenital endometriosis,
thereby facilitating multidisciplinary collaboration.
These guidelines were originally written in Japanese,and this English version was translated from the Japanese version.4

information provided by the NPO Japan Medical
Abstract Society. English or Japanese keywords, listedfrom each PICO formatted clinical questions, wereused for the literature search.
The systematic review was performed by an initialand secondary screening of the literature identiﬁed by
the Japan Medical Library Association. We evaluatedand integrated evidence from the systematic reviewof these articles and wrote the Systematic Review
Reports. The strength of the supporting evidence wasscored as A (strong), B (moderate), C (weak) or D(very weak), according to the Medical Information
Network Distribution Service guideline and the Grading of Recommendation, Assessment, Development,
and Evaluation systems.5 Furthermore, the recommendations and recommendation levels were
established by all members of the CPG committee
using modiﬁed Delphi methods. The strength of therecommendation was classiﬁed as 1 (strong) or
2 (weak). After ﬁnalization of the guideline draft, it
was submitted to the Japan Society of Obstetrics andGynecology (JSOG) and the Japan Society of Endometriosis (JSE) for external review and evaluation andfor collecting public opinion. The CPG draft wasrevised accordingly, and the ﬁnal version wasapproved by the CPG committee, JSOG and JSE.

Clinical Questions andRecommendations
Procedure of Developing the Guidelines
The clinical practice guideline (CPG) committee wasformed by the research team studying extragenital
endometriosis, which is supported by the research
program for intractable disease of the Japanese Ministry of Health, Labor and Welfare. The members of
CPG committee included experts of gynecology, gastrointestinal surgery, urology, respiratory surgery andplastic surgery. The CPG committee formulated eightclinical questions for the treatment of intestinal, bladder/ureteral, thoracic and umbilical endometriosis,
structured by the PICO (P: patients, I: intervention, C:
comparisons, O: outcomes) format. For each question,
a literature search was performed by the Japan Medical Library Association (http://plaza.umin.ac.jp/~jmla/eng/index_eng.html), a nonproﬁt organization, using the PubMed, Cochrane and Ichushi databases covering the period from January 2006 to
November 2016. The Ichushi database is an Internetbased retrieval service for Japanese medical article

The summary of clinical questions and recommendations is shown in Table 1.
CQ 1: Is medical treatment recommended forintestinal endometriosis?
Recommendation:• Medical therapy is recommended for rectosigmoid
endometriosis because it is effective in improving
symptoms and reducing the size of lesions. (1C)
• The efﬁcacy of medical therapy for intestinal endometriosis other than the rectosigmoid (ileocecal
region, appendix, small intestine) is unknown. (2D)Literature search and screening
For this CQ concerning intestinal endometriosis,
33 English articles and 35 Japanese articles were
found by searching the PubMed, Cochrane andIchushi databases. In the ﬁrst screening, 17 English© 2020 The Authors. Journal of Obstetrics and Gynaecology Research published by John Wiley & Sons Australia, Ltdon behalf of Japan Society of Obstetrics and Gynecology.

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T. Hirata et al.

Table 1 Summary of clinical questions and recommendations#

Clinical question

Recommendation

Strength ofrecommendation

Strength of
supporting
evidence1

Is medical therapy recommended
for intestinal endometriosis?

Medical therapy is recommended
for rectosigmoid endometriosis
because it is effective in
improving symptoms andreducing the size of lesions.
The efﬁcacy of medical therapy
for intestinal endometriosis
other than the rectodigmoid(ileocecal region, appendix,
small intestine) is unknown.
Surgical treatment isrecommended for symptomatic
intestinal endometriosis that is
difﬁcult to control with medical
therapy.
Medical therapy is effective andrecommended for treating
bladder endometriosis.
Medical therapy may be lesseffective for ureteralendometriosis withhydroureteronephrosis.
Surgical treatment for bladder
endometriosis/ureteralendometriosis may be effective
depending on the case andoperative method.
Surgical treatment for catamenial
pneumothorax may be effective
depending on the symptoms.
Catamenial hemoptysis may beameliorated by conservative
treatment without surgery,but surgery is to beconsidered when symptoms
are severe.
As for thoracic endometriosis,
medical therapy, alone or as
postoperative adjuvant therapy,
may be considered, dependingon the case.
For umbilical endometriosis,
radical surgery with wide local
excision is recommended,
depending on the case.
Medical therapy can be an option
for umbilical endometriosis.

1

C

2

D

1

C

1

C

2

C

1

C

1

C

2

D

2

C

1

C

2

D

2

Is surgical therapy recommended
for intestinal endometriosis?

3

Is medical therapy recommended
for bladder endometriosis/
ureteral endometriosis?

4

Is surgical therapy recommended
for bladder endometriosis/
ureteral endometriosis?

5

Is surgical therapy recommended
for thoracic endometriosis?

6

Is medical therapy recommended
for thoracic endometriosis?

7

Is surgical therapy recommended
for umbilical endometriosis?

8

Is medical therapy recommended
for umbilical endometriosis?

Strength of recommendation was classiﬁed as 1 (strong) or 2 (weak). Strength of supporting evidence was scored as A (strong), B (moderate), C (weak) or D (very weak).

articles and 17 Japanese articles were selected. In thesecond screening, eight English papers and one Japanese paper were used to provide a response tothis CQ.
2476

Of the nine studies, one was a systematic review,6seven were prospective cohort studies7–13 and one
was a retrospective study.14 All articles focused onrectosigmoid endometriosis, and there were no© 2020 The Authors. Journal of Obstetrics and Gynaecology Research published by John Wiley & Sons Australia, Ltdon behalf of Japan Society of Obstetrics and Gynecology.

Guideline for extragenital endometriosis

articles concerning endometriosis of the ileocecal
region, small intestine or appendix.
Review and comment

reported any serious adverse effects or complications.
Note that almost all these reports examined the effectof medical therapy within 1 year, and no report to datehas examined the long-term prognosis such as therecurrence rate after discontinuation of the therapy.

1. Rectosigmoid endometriosis
With regard to the outcomes, eight studies6–10,12–14
evaluated symptom and some of these studies6,10,11,14
evaluated the lesion size. However, comparisons were
not conducted between groups with and without
medical therapy, but before and after medical treatment, or between several medicines. In addition, ﬁveof the seven prospective cohort studies were from thesame institution in Italy,7–9,11,12 which could represent
a bias.
A systematic review of 217 cases from 7 papers
reported that 6–12 months of vaginal danazol, GnRH
agonist, progestin-releasing intrauterine system andoral contraceptives (OC) were all effective in relieving
pain.6 Among seven prospective cohort studies, one
evaluated the size of the lesions as an outcome, andreported that 12-month of norethisterone, GnRH analog plus tibolone add-back therapy, norethisterone
plus letrozole, desogestrel and OC were all effective
in reducing the size.11 Four studies evaluated thesymptoms as an outcome. GnRH analog plus estradiol add-back therapy for 3 months,13 GnRH analog
plus tibolone add-back for 12 months,9 norethisterone
plus letrozole for 6 months7 and norethisterone monotherapy for 12 months8 were all effective in improving symptoms, compared to their pre-treatment
conditions. Two studies evaluated both the symptoms
and the size of the lesions as outcomes. One study
compared a 12-month estrogen/progestin vaginal
ring with orally administered desogestrel, and concluded that both treatments were effective in pain
relief and reducing lesion sizes, and that patient satisfaction was signiﬁcantly higher with oral desogestrel.12 The other study reported that continuous
administration of OC for 12 months was effective in
reducing symptoms and lesions compared to before
treatment.10
In a retrospective study of 82 cases of intestinal
endometriosis, 55 patients (67.1%) underwent hormonal therapy (OC, dienogest, GnRH agonist or danazol), and 43 cases (78.2%) showed improvement insymptoms.14 In particular, dienogest was highly effective in reducing lesion sizes and improving symptoms.
Although no detailed studies have been conductedexamining adverse effects, none of the articles

2. Intestinal endometriosis of the ileocecal region,
small intestine, and appendix
A review of the literature revealed that a few casereports and case series have been published and there
are currently no reports assessed on the efﬁcacy of
medical treatment for these rare types of intestinal
endometriosis.
Summary
For rectosigmoid endometriosis, medical therapy may
be useful for improving symptoms and reducing lesion
sizes. Given the high complication rate of surgery, medical treatment may be the ﬁrst option, although there isno evidence of superiority to surgery. For intestinal
endometriosis other than for rectosigmoid (e.g., ileocecal,
appendix, small intestine), there is no available evidenceof the efﬁcacy of medical treatment, or its superiority to
study. Medical therapy can be an option for this raretype of intestinal endometriosis; however, when thesymptoms are not controlled by medical treatment, surgical therapy should be considered.
The adverse effects and complications of medical
therapy for intestinal endometriosis may be comparable to those used for pelvic endometriosis, such as
ovarian endometrioma, although unique complications of medical therapy when used for intestinal
endometriosis are unknown. The recurrence rate after
discontinuation of medication is also unknown, but
long-term management may be required, as in thecase of endometriosis at other sites.
CQ 2: Is surgical therapy recommended for intestinal endometriosis?
Recommendation:• Surgical treatment is recommended for symptomatic intestinal endometriosis that is difﬁcult to control with medical therapy. (1C)
Literature search and screening
For surgical treatment of intestinal endometriosis,
246 English and 251 Japanese articles were identiﬁed© 2020 The Authors. Journal of Obstetrics and Gynaecology Research published by John Wiley & Sons Australia, Ltdon behalf of Japan Society of Obstetrics and Gynecology.

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T. Hirata et al.

by searching the PubMed, Cochrane and Ichushi databases. At the ﬁrst screening of these publications,
123 English papers and 5 Japanese papers wereselected, and in the second screening, 19 English
papers were selected to answer this CQ.
Of these 19 studies, 13 were case series,15–27 4 werecohort studies28–31 and 2 were randomized controlled
trials (RCT).32,33
Review and comment
The four cohort studies28–31 and the two RCT32,33
compared different surgical methods, and did notcompare a surgically treated group with a non-treatedgroup. Five case series,15–19 1 cohort study (ablative
surgery with [study group] or without bowel resection [control group]),28 and one RCT (laparotomy
vs laparoscopic surgery) examined postoperative
improvement of symptoms.32 In all studies, surgicaltreatment showed improvement in symptoms.
Regarding surgical complications, three cohort
studies28,30,31 and two RCT32,33 did not report thedetails of the complications; thus, the frequency ofserious complications such as anastomotic insufﬁciency and rectovaginal ﬁstula are unknown. Elevencase series15–18,20–26 and one cohort study (nervesparing surgery vs conventional surgery)29 describedpostoperative complications in detail.
In four case series reports,15–18 the authors examined postoperative symptom improvement. In one
case series,19 one cohort study28 and one RCT32 symptom improvement was evaluated by scoring. All studies suggested that surgical treatment improved
symptoms. Therefore, although the level of evidence
is low, surgical treatment may improve symptoms.
With regard to complications, the frequency of serious complications such as anastomotic insufﬁciency,
rectovaginal ﬁstula and dysuria was investigated in
11 case series,15–18,20–26 and in 1 cohort study.29 Anastomotic insufﬁciency, rectovaginal ﬁstula and dysuria
were reported in 0–2%, 1.8–4% and 0.8–29% of cases,
respectively. Since serious complications have beenreported, the indications for surgery should bedeﬁned considering the risk of complications.
Summary
Among the 19 papers reviewed, there were noreports comparing surgically treated cases with nonsurgically-treated cases, and there is no literature
available that directly answered this CQ. However,2478

according to the literature, surgical treatment can
improve or relieve symptoms. Therefore, surgicaltherapy is an option for symptomatic intestinal endometriosis that is difﬁcult to control with othertherapies.
CQ 3: Is medical therapy recommended for bladder endometriosis/ureteral endometriosis?
Recommendation:• Medical therapy is effective and recommended fortreating bladder endometriosis. (1C)
• Medical therapy may be less effective for ureteralendometriosis with hydroureteronephrosis. (2C)
Literature search and screening
We identiﬁed 19 English articles and 22 Japanese
articles investigating medical therapy for bladder
endometriosis and ureteral endometriosis bysearching the PubMed, Cochrane and Ichushi databases. Eleven English articles and eight Japanese articles (all English articles were case reports and caseseries) were selected for the primary screening. In thesecondary screening, four English articles (one systematic review, two reviews and one case series) wereselected as the ﬁnal references. One systematic review
included ﬁve English papers. Of the four English
papers, two were about bladder endometriosis34,35
and one was a review of ureteral endometriosis.36
These reviews included case reports and case series
excluded by screening.
There were no RCT, meta-analyses or cohort studies. There was one report of ureteral endometriosis
treated with aromatase inhibitors,37 but it was notincluded in the above three articles and was added tothe ﬁnal article list.
Review and comment
1. Bladder endometriosis
In a systematic review of the literature on bladder
endometriosis, 36 patients were included in 9 casereports describing medical therapy.34 The authors
suggested that OC and dienogest were the ﬁrst choicetreatments, and GnRH agonists were the second
choice, both of which require long-term treatment.
Administration of dienogest for 6 months or more
had the effect of reducing lesions and improving© 2020 The Authors. Journal of Obstetrics and Gynaecology Research published by John Wiley & Sons Australia, Ltdon behalf of Japan Society of Obstetrics and Gynecology.

Guideline for extragenital endometriosis

symptoms. It has also been pointed out that GnRH
agonists have a stronger effect on lesion reduction
than OC. Considering that aromatase inhibitors areoften ineffective, have side effects such as muscle pain
and joint pain, and are still a focus of clinical research,
they should be considered as an option when the ﬁrst
and second choice treatments are ineffective. It has
been pointed out that all medical therapies have a
temporary effect during treatment.
Another article reviewed 23 studies on medical
therapy for bladder endometriosis reported between
1996 and 2011.35 In addition to the GnRH agonists,
dienogest and OC, this review suggested the efﬁcacy
of medal progesterone acetate depot and danazol
treatment.
Medical therapy was most suitable for patients withsmall endometriotic lesions of ≤5 mm in size, but
long-term administration is required because therecurrence rate after discontinuation of medication ishigh at 35%.
Ureteral endometriosis
One article reviewed two reports on medical therapy
for ureteral endometriosis reported from 1996 to 2010.36
The review showed that the course of hormonal therapy
may be similar to that of bladder endometriosis, that
medical treatment is effective, and that ultrasound examination every 6 months could assess the development ofureteral obstruction during treatment. Hormone therapy
appears to be effective in suppressing the growth of ureteral endometriotic tissue, but has modest effects onﬁbrotic lesions and scars. Furthermore, it has been
pointed out that hormonal treatment alone has a poor
effect on lesions with ureteral obstruction due to adhesion
with surrounding tissues. In addition, there was a casereport describing a patient with bilateral ureteral endometriosis receiving aromatase inhibitor.37 Another report
describing a patient who received a GnRH agonist for
6 months, and subsequently an aromatase inhibitor for
15 months, reported that ﬁbrotic lesions were not
reduced, and the patient eventually required surgicaltreatment.
Summary
For patients with bladder endometriosis, medical
therapy can be the ﬁrst choice because it can be
expected to relieve symptoms. For ureteral endometriosis, the same medical therapy for bladder endometriosis may be given. However, when ureteral stenosis iscaused by a ﬁbrotic lesion, the therapeutic effect ispoor. Therefore, it is necessary to carefully select suitable patients for treatment. In any urinary tract endometriosis, medical treatment can be expected torelieve symptoms and reduce lesions, but long-term
therapy is required.
CQ 4: Is surgical therapy recommended for bladder endometriosis/ureteral endometriosis?
Recommendation:• Surgical treatment for bladder endometriosis/ureteral endometriosis may be effective depending on
the case and operative method. (1C)
Literature search and screening
First, for this CQ concerning bladder/ureteral endometriosis, 228 English documents and 122 Japanese
documents were searched using the PubMed,
Cochrane and Ichushi databases. A total of 102 English
papers were selected for the primary screening, and
20 English papers were selected for this CQ in the secondary screening.
No RCT has examined the efﬁcacy of surgical therapy. Of the 20 articles identiﬁed, 4 were prospective
case series38–41 and only 1 was a prospective study
with comparison of surgical treatment.38 Sixteen studies retrospectively summarized surgical cases for
bladder and ureteral endometriosis,42–57 most ofwhich involved laparoscopic surgery and one
involved robot-assisted surgery.45
Review and comment
To answer this CQ, a comparative study of surgical treatment and non-surgical treatment is necessary. In addition, prospective RCT are essential
because of the potential impact of various factors
such as the severity of endometriosis, patient age,
symptoms, and pretreatment administered. The
answer depends greatly on the criteria deﬁned as‘effective’. For example, postoperative recurrence
rate, reoperation rate, complications, alleviation ofsymptoms, improvement of reproductive function,
improvement of urinary function, improvement ofhydronephrosis, and so on are assumed to be thecriteria of ‘effectiveness of surgical treatment’. However, no valid results have been reported for thesestudies.

© 2020 The Authors. Journal of Obstetrics and Gynaecology Research published by John Wiley & Sons Australia, Ltdon behalf of Japan Society of Obstetrics and Gynecology.

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Generally, surgical treatment for bladder endometriosis/ureteral endometriosis includes ureterolysis,
ureteroureterostomy, ureterocystoneostomy, transurethral resection of endometriosis,54 partial cystectomy
and nephrectomy, each of which may be performedby open surgery, endoscopic surgery, or roboticassisted surgery. In addition, ureteroscopic ablation
with a holmium YAG laser has also been reported.46
In a prospective case series of 56 cases of ureteralendometriosis, Mereu et al. performed laparoscopic
ureterolysis (35 cases), laparoscopic ureteroureterostomy(17 cases), laparoscopic ureterocystoneostomy (2 cases)
and laparoscopic nephrectomy (2 cases).38
Comparing the 35 cases of laparoscopic ureterolysis
and 17 cases of laparoscopic ureteroureterostomy,
ureterolysis cases presented signiﬁcantly more complications, during a median follow-up period as short
as 21 months. Soriano et al. reported that among 41 of
45 patients (91.1%) that underwent laparoscopic
ureterolysis, only 2 (4.4%) needed reoperation.39 In
addition, Seracchiolo et al. reported that 22 of 30 patients(73.3%) underwent ureterolysis and 8 patients (26.7%)
had recurrence of endometriosis during the averagefollow-up period of 55 months.41 As described above,the evaluation of efﬁcacy may vary greatly dependingon the type of surgical therapy. If limited to ureteralendometriosis, ureteral stent placement is a surgicaltreatment option and needs to be evaluated.
Accordingly, there are variations in the requirements
for the selection of surgical procedures for bladder endometriosis and ureteral endometriosis, and thus the results
may be biased. The choice of surgical therapy and theactual surgical procedure are strongly affected by thepatient’s age, clinical condition (particularly reproductive
and renal function), or the stricture site and extent of urinary tract involvement.39 There is not enough evidence torespond to this CQ, as there are no studies comparing
surgery to other therapies and only results from surgery
alone have been reported. However, surgery may beeffective because there are consistent reports of symptom
improvement and low postoperative recurrence rate incase series. Further studies are also needed on the effectiveness of medical treatment as a prior treatment/combination therapy. In the future, it would be desirable toestablish an algorithm for selecting treatment methods,
including surgical therapy.
Summary
For this CQ, there is no clear evidence from caseseries reports. However, considering reports of each2480

case series, surgical treatment for bladder endometriosis/ureteral endometriosis may be effective, and further study is required to provide an answer tothis CQ.
CQ 5: Is surgical therapy recommended for thoracic endometriosis?
Recommendation:• Surgical treatment for catamenial pneumothorax
may be effective depending on the symptoms. (1C)
• Catamenial hemoptysis may be ameliorated byconservative treatment without surgery, but surgery is to be considered when symptoms aresevere. (2D)Literature search and screening
To determine the efﬁcacy of surgical treatment of
thoracic endometriosis, 75 articles in English wereidentiﬁed by searching the PubMed, Cochrane andIchushi databases. Twenty-four English articles
were selected during the ﬁrst screening, and 13 articles in English were selected as references for thisCQ in the second screening. Among these 13 articles, 11 were on catamenial pneumothorax,58–68
1 was on catamenial hemoptysis69 and anotherdescribed both conditions.70 One was a multicenter
study investigating catamenial hemoptysis, 70
10 studies were reports of single-center case studiesof thoracic endometriosis,58–66,70 and 2 werereviews of previous articles on catamenial pneumothorax.67,68 Nine articles on catamenial pneumothorax were retrospective observational studies
including 4–150 cases,58–66 4 of which revealed thefrequency of catamenial pneumothorax among
spontaneous pneumothorax in women.58,61,63,64 On
the other hand, catamenial hemoptysis is a rare disease, and approximately 40 cases have beenreported in English. The 19 cases reported in a single multicenter study described the clinical features, imaging ﬁndings, treatment and surgicaloutcomes of catamenial hemoptysis, but there wasno comparison with other treatments.69 The literature discussing surgical treatment of catamenial
pneumothorax described its clinical features, operative procedure and hormonal treatment, and evaluated the pneumothorax recurrence rate as theoutcome. Two reviews were on catamenial
pneumothorax,67,68 and one article discussed thesigniﬁcance of serum CA 125 levels as a diagnostic
approach. 66© 2020 The Authors. Journal of Obstetrics and Gynaecology Research published by John Wiley & Sons Australia, Ltdon behalf of Japan Society of Obstetrics and Gynecology.

Guideline for extragenital endometriosis

Review and comment
As a surgical method for spontaneous pneumothorax, it is common to thoracoscopically suture orresect the lesions of the visceral pleura that causes
air leakage. Even in the case of catamenial pneumothorax, in the acute phase, the endometriotic lesionthat causes air leak should be resected to stop the
air leak. In addition, if there are defects in the diaphragm, which are suspected to be the cause of theinﬂow of the endometrium into the thoracic cavity,
it should be closed. Although pleurodesis may be
performed to prevent recurrence of pneumothorax
due to the onset of pleural lesions, it is not clear
whether pleurodesis should be performed. The
recurrence rate was deﬁned as the outcome of thecase series described in 10 reports of catamenial
pneumothorax.58–66,68 The details of the surgicalprocedure
weredescribedinsevenreports,58,60,62,64–66,70 and postoperative hormonal
therapy was described in ﬁve reports.58,61,62,65,70
None of the articles compared surgical therapy
with other treatments and did not provide a clear
answer to this CQ.
Summary
Of the 13 papers reviewed for thoracic endometriosis, there was no evidence available to answer
to this CQ. The clinical features, histopathologicalﬁndings and postoperative recurrence rate of catamenial pneumothorax and hemoptysis were analyzed in 12 58–68,70 and 2 case series reports, 69,70
respectively. If catamenial pneumothorax is very
severe, it may cause respiratory failure and may
be life-threatening. Therefore, it is recommended
to determine the surgical indication based on
spontaneous pneumothorax. For catamenialhemoptysis, surgical therapy may be considered if
the condition is severe to cause airway obstruction
due to a clot.
CQ 6: Is medical therapy recommended for thoracic endometriosis?
Recommendation:• As for thoracic endometriosis, medical therapy,
alone or as postoperative adjuvant therapy, may beconsidered, depending on the case. (2C)

Literature search and screening
For this CQ, 111 English and 15 Japanese studies
were identiﬁed from the PubMed, Cochrane, andIchushi databases. Eighteen English articles andeight Japanese articles were selected for the primary screening, and eight English articles andthree Japanese articles were selected as references
for this CQ in the second screening. Of these
11 articles, 10 were case series 65,70–78 and 1 was a
retrospective cohort study. 62 Seven reports wereon catamenial pneumothorax, 65,72–74,76–78 threewere on treatment experiences for thoracic endometriosis 62,70,71 and one was related to adverse
effects of medical therapy. 75 Of the case series
associated with medical therapy, surgical intervention was performed in all cases series except
for one report, and even in this remaining one, 70
there was no comparison between outcomes of
medical therapy alone group and the control
group. One cohort study investigated on
pleurodesis and on the recurrence of catamenial
pneumothorax after the treatment with or without
postoperative adjuvant hormonal therapy.62
Review and comment
Ten reports65,70–78 were reviews of a large numberof cases treated for thoracic endometriosis and did
not compare the medical treatment group including
pleurodesis with the control group. Regarding therecurrence of clinical symptoms after treatment, one
paper reported cases treated pharmacologically(GnRH agonist alone or GnRH agonist + dienogest)or surgically,70 and eight papers reported cases
treated with or without postoperative hormonal
therapy,65,71–74,76–78 two of which included cases ofrecurrence after surgery with postoperative dienogest
therapy.76,77 One was a case series reporting theadverse effects of medical therapy.75 No study investigated the effects of medical treatment, including
pleurodesis, on the recurrence of catamenial pneumothorax. Therefore, the available evidence is not sufﬁcient to answer this question. One retrospective
cohort study reported recurrence of symptoms withand without pleurodesis and postoperative adjuvant
drug therapy (GnRH agonist alone), but no control
group was used.62 None of the reports described theassociation between lesion size and the effects of medical therapy.

© 2020 The Authors. Journal of Obstetrics and Gynaecology Research published by John Wiley & Sons Australia, Ltdon behalf of Japan Society of Obstetrics and Gynecology.

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Summary
Of the 11 articles reviewed, there was no available
literature that provided evidence to directly answer
this CQ. However, GnRH agonist alone or GnRH
agonist–dienogest sequential administration may beconsidered as effective medical therapy.
CQ 7: Is surgical therapy recommended for umbilical endometriosis?
Recommendation:• For umbilical endometriosis, radical surgery withwide local excision is recommended, depending on
the case. (1C)
Literature search and screening
A literature search of the PubMed, Cochrane andIchushi databases retrieved 107 English papers and
32 Japanese papers. In the ﬁrst screening of these documents, 47 English and 19 Japanese studies wereselected, and in the second screening, 13 Japanese79–91
and 33 English92–124 were selected as relevant articles
for this CQ.
Since there has been no prospective comparative
study on the efﬁcacy of treatment for umbilical endometriosis, we examined case reports and case series.
Review and comment
Of 89 cases of umbilical endometriosis, 82 weretreated with surgical therapy and 7 were conservatively treated with medical therapy alone. Considering that the symptoms were improved by surgery,and no speciﬁc complications were reported, surgicaltreatment may be effective in improving symptoms.
In most cases, the postoperative follow-up period wasless than 6 months or was not speciﬁed. Recurrent
patients had relapsed more than 2 years after surgery.
Therefore, surgical treatment may be effective in the
short term, but the long-term recurrence rate andcomplications remain unknown.
In order to avoid recurrence after surgical therapy, it is preferable to excise the lesion to include alarge portion of marginal tissue (wide local resection). Umbilical deformities associated with wide
local resection may occur and sometimes require
umbilical reconstruction by plastic surgeons.
Accordingly, surgical therapy may be effective in
2482

improving or relieving symptoms, at least in the
short term.
Saito et al. reported seven cases of umbilical endometriosis, in which three patients were followed up
without treatment, three were treated with OC and
one was treated with surgery.113 Their results
suggested that OC treatment is effective in alleviating
symptoms. It can be recommended for patients withmild symptoms who do not wish to get pregnant.
Conversely, discontinuation of medication often causes relapse of symptoms, and surgical treatment isstill necessary as radical treatment.
The etiology of umbilical endometriosis is still
unclear, but two types have been described. The ﬁrst
is a secondary umbilical endometriosis that arises onscar tissue following abdominal surgery such as laparoscopic surgery, and the other is primary umbilicalendometriosis that occurs in the absence of surgicalhistory. Of the 89 cases of umbilical endometriosis
described, 16 cases were secondary and 73 cases wereprimary. Although there is no literature comparing
the therapeutic effects of surgical treatment for primary and secondary umbilical endometriosis, it may
possibly affect the therapeutic outcome.
Summary
A review of the literature suggests that surgicaltreatment may have short-term efﬁcacy for umbilicalcord endometriosis, but long-term efﬁcacy and complications remain unknown. Although no study has
directly compared medical therapy and surgical therapy, surgical therapy should be the ﬁrst choice since
the umbilicus is easy to approach and the invasiveness of surgery is relatively minimal.
CQ 8: Is medical therapy recommended for umbilical endometriosis?
Recommendation:• Medical therapy can be an option for umbilicalendometriosis. (2D)Literature search and screening
Seven English and 10 Japanese articles were identiﬁed for the CQ of umbilical endometriosis. Seven
English and eight Japanese studies were selected in
the ﬁrst screening, and seven English papers were

© 2020 The Authors. Journal of Obstetrics and Gynaecology Research published by John Wiley & Sons Australia, Ltdon behalf of Japan Society of Obstetrics and Gynecology.

Guideline for extragenital endometriosis

selected as the ﬁnal references in the second screening. All seven papers were case reports, two of which
included case reports and a review of previously
reported cases.124,125
Review and comment
All reports except one were case reports describing
surgical treatment, and the description of the effect of
medical therapy was limited. Since surgical treatment
is the major treatment for umbilical endometriosis,
there has been no literature evaluating the efﬁcacy of
medical therapy alone.
In one case series, medical therapy was reported tobe effective in improving symptoms in three cases,
but the case series was not a study comparing with/without medical therapy or different agents.113 Therefore, it is difﬁcult to answer not only the efﬁcacy of
medical therapy alone, but also comparison with surgical therapy, comparison between different medicines and complications.
In a review of 231 reported cases, only 3 received
medical therapy alone and 228 underwent surgicaltreatment.124 The authors noted that although medical
therapy is useful for relieving symptoms, the administration of medical therapy is likely to be prolonged
and the disease may recur after discontinuation of
medication. Before choosing medical therapy, it isnecessary to exclude malignant diseases. In a caseseries of seven patients, an OC was administered tothree patients, and their symptoms improved.113
Although symptoms recurred when hormone therapy
was discontinued, hormone therapy has beenreported to improve symptoms and can be an adjunctive postoperative treatment to prevent recurrence, if
the endometriotic lesions are positive for estrogen andprogesterone receptor expression.118
According to another study, hormone therapy isless effective for endometriosis that develops in theabdominal wall or that arises from surgical scar tissues, and symptoms tend to relapse when the medication is discontinued, but medical therapy is an option
if concurrent severe pelvic endometriosis is present.102
In addition, preoperative administration of GnRH
agonists is not recommended because of the increased
risk of incomplete resection due to reduced tumor sizeand difﬁculty in its localization. In another study,
approximately 70% of umbilical endometriosis wastreated surgically, and hormonal therapy may havebeen effective in reducing tumor size and improving
symptoms, although the effect was not certain.101

Furthermore, preoperative treatment with dienogest
and OC may be effective, but it is difﬁcult to cure theendometriosis with hormone treatment alone, while
surgical treatment is preferable to conservative treatment because of complete surgical excision and histological conﬁrmation.96
None of the available literature has reported any
serious adverse effects or complications of medical
therapy.
Summary
The reported number of cases of umbilical endometriosis was small, and there were no high-level evidence articles. Based on the literature review, surgicaltreatment is the main treatment for umbilical endometriosis, and few cases have received medical therapy.
Therefore, it was difﬁcult to answer thisCQ. Furthermore, there have been no studies comparing medical and surgical therapy, comparing different
agents, reporting the frequency of adverse effects/
complications, or the recurrence rate after treatment
discontinuation. Further studies are required toanswer this CQ.

Acknowledgments
This work was supported by grants from the Ministryof Health, Labour and Welfare. The authors would
like to thank Editage (www.editage.jp) for the English
language review.

Disclosure
None declared.

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