Introduction
Thoracic endometriosis syndrome (TES) is one of the extrapelvic forms of endometriosis,
and it can involve the airways, pleura and lung parenchyma. This rare condition includes
four entities: catamenial pneumothorax, catamenial hemothorax, catamenial hemoptysis
and lung nodules.[1]
[2]
Considered an extremely exceptional condition, the TES diagnosis is repeatedly delayed
and, many times, confounded with other diseases, mainly when it is presented as hemoptysis,
which is even rarer. The literature evidence about TES is very sparse and most of
the findings come from case reports, which suggests an important limitation regarding
the data.[2]
[3]
Cyclic manifestations of pulmonary diseases, such as chest or shoulder pain, dyspnea
and hemoptysis, which occur along with the menstrual period, should be considered
as an alert for any gynecologist or clinician. In such cases, the hypothesis of TES
should be seriously considered, and a further investigation with imaging techniques
ought to be implemented for diagnosis, despite of nonspecific findings.[4]
Clinical management, due to its conservative character, should be considered as the
first-line therapy, and the surgical approach must be cogitated when the pharmacological
approach fails.[5]
[6]
Case report
A 23-year-old woman presented with complaints of hemoptysis during the menstrual period,
associated with pain in the right shoulder for the past 2 years, with no prior background
of smoking or tuberculosis. At admission, her clinical examination and chest X-ray
showed no abnormality. She was clinically treated for tuberculosis for several times;
however, there was no remission of the symptoms.
Taking into consideration that the hemoptysis occurred only during menses, an inquiry
about endometriosis was performed, and the patient revealed that dyspareunia and dysmenorrhea
were recurrent.
Further investigation with computed tomography (CT) scans during menses showed an
area of ground glass haze in the upper lobe of the right lung ([Fig. 1]) and for a better inquiry, a bronchoscopy was performed between menses, and the
bronchial washing turned to be negative. As a complementary investigation, the patient
underwent transvaginal ultrasonography to investigate the presence of pelvic endometriosis,
which was a positive finding. Endometriosis was apparently affecting the posterior
fornix and the sacral-uterine ligament. Considering the emergence of symptoms during
menses, an empirical treatment with Dienogest (2mg/day) was established.
Fig. 1 An area of ground glass haze in the upper lobe of the right lung.
The first follow-up took place two months after the beginning of therapy, and the
patient was clinically asymptomatic. A control CT scan was performed and revealed
a current recovery of the affected area ([Fig. 2]). After the one-year follow-up, the patient remains clinically asymptomatic.
Fig. 2 Recovery on the affected area (computed tomography image without an area of ground
glass haze in the upper lobe of the right lung).
Discussion
Endometriosis is an estrogen-dependent inflammatory disease that is pronounced by
the presence of endometrial tissue outside the uterus, affecting several structures
and organs, such as the rectosigmoid colon, rectovaginal septum, uterosacral ligament,
bladder and even extrapelvic organs.[7]
Thoracic endometriosis syndrome (TES) is one of the extrapelvic forms of endometriosis,
and it can involve the airways, pleura and lung parenchyma. This rare condition includes
four entities: catamenial pneumothorax, catamenial hemothorax, catamenial hemoptysis
and lung nodules.[1]
[2]
The most usual appearance of TES is catamenial pneumothorax (73%), and the rarest
is catamenial hemoptysis, jointly with lung nodules (6% each), as we see in our patient
who presents both nodule and hemoptysis.[5]
[8]
Many theories attempt to clarify the presence of extrapelvic endometrial implants,
including intrathoracic implants. Among these theories is coelomic metaplasia, which
is based on the hypothesis that both endometrium and pleura have the same embryologic
origin, and pathogenic incitements could induce precursor cells to differentiate into
endometrial cells.[7]
Another widespread theory, lymphatic or hematogenic dissemination, would also elucidate
the occurrence of extrapelvic endometriosis, including TES, and its propensity to
the right lung since the right lymphatic drainage system is more complex than the
left hemithorax.[2]
[7]
Clinical manifestation of TES is exceedingly uncommon, and most of the data on this
syndrome come from case reports and a retrospective study including 110 patients.
Joseph J. et al showed that the mean age at the onset of symptoms was 35 years old
and the peak incidence for TES occurred ∼ 5 years after the incidence of pelvic endometriosis
in these patients.[8]
Contrary to what the literature shows, our patient presented both symptoms, pelvic
pain and hemoptysis, at the same time, and the main symptom presented by our patient,
cyclical hemoptysis, an extremely rare condition, has been described in ∼ 30 cases
in the literature.[9]
Thoracic endometriosis syndrome is considered a thought-provoking diagnostic due to
its undefined symptoms, and there is an enormous delay until the disclosure of the
diagnosis. Cyclical and recurrent complaints, such as shoulder and chest pain, hemoptysis
and dyspnea, mainly when related with pelvic endometriosis background, ought to ring
a bell about TES.[5]
[10]
Not only clinical but also imaging aspects are open-ended, even though CT endures
as the first-line imaging exam, enabling exclusion of many other lung diseases with
similar symptoms and also assisting with the diagnosis in patients with significant
clinical description. Magnetic resonance imaging is considered superior to CT, as
it can notice blood and its products when performed during menses.[5]
[10]
Bronchoscopy has a limited role in diagnosis, since most pathologic features are situated
in the areas around the lung; as we see in our case reported, the bronchoscopy was
completely normal. Regarding video-assisted thoracoscopy, a direct imagining of lung
parenchyma, pleura and diaphragmatic shell offers a precise diagnostic.[2]
Considering that there is no definitive treatment for endometriosis, in general, one
has to cogitate options for a long-term management. Frequently, hormonal overthrow
is the first option in the attempt to control symptoms caused by endometriosis and
to postpone or even avoid surgical treatment due to great morbidity.[11]
[12]
[13]
Surgical management by thoracoscopy, or even thoracotomy, aims to remove all the endometriotic
tissue and sometimes, a pulmonary segmentectomy is required. This treatment is regarded
when clinical approach fails.[6]
[7]
[8]
[9]
[10]
[11]
[12]
[13]
[14]
A variety of drugs has been used to treat endometriosis, including TES, such as combined
oral contraceptives, progestogens and gonadotropin-releasing hormone (GnRH) agonists,
and all of them have presented comparable usefulness. Safety, acceptability and cost
need to be considered in order to implement a long-term treatment. [11]
Dienogest, a fourth-generation progestogen, has been reported as an effective drug
in the treatment of complaints caused by endometriosis, acting through inhibition
of GnRH, inducing estrogen deprivation and causing decidualization and atrophy of
endometriotic lesions with few and very tolerable side effects.[12]
[15]
As we see in the case reported, after the treatment with dienogest (2 mg/day), our
patient remained completely asymptomatic and had no further episodes of hemoptysis
or pelvic pain.
In conclusion, TES is a rare disorder that is commonly missed or whose diagnosis is
delayed. It may be suspected in women with symptoms during the menstrual period. Computed
tomography scan has an important role in presumptive diagnosis. Surgical treatment
is an option in some cases; however, the clinical treatment is very effective and
suppresses endometriotic tissue growth.
