INTRODUCTION
Endometriosis is characterized by the presence of endometrial tissue outside the
uterine cavity1. This condition affects
approximately 12% of women of reproductive age2, and the primary sites of involvement are the ovaries, sacrum,
broad ligaments of the uterus, uterosacral ligaments, fallopian tubes, sigmoid
colon, appendix, and round ligaments of the uterus1. Umbilical endometriosis represents 0.5% to 1% of all cases2. Malignant complications are rare,
corresponding to 1% of all cases3.
In this study, we describe a patient with umbilical endometriosis, and review the
diagnosis and treatment.
CASE REPORT
A 35-year-old woman was referred to the Plastic Surgery Department of the São
Joaquim Hospital of Franca, Franca, São Paulo state, Brazil, with umbilical
vegetative lesions that appeared 6 months prior. Physical examination indicated
the presence of a primary lesion that was painful to palpation and surrounded by
2 smaller lesions, all of which were associated with bleeding and pain during
the menstrual period (Figure 1). Computed
tomography of the abdomen showed an umbilical nodule compatible with probable
umbilical endometriosis (Figure 2).
Figure 1 - Patient with vegetative umbilical lesions. Surgical scar due to
previous piercing. Vegetative umbilical lesions.
Figure 1 - Patient with vegetative umbilical lesions. Surgical scar due to
previous piercing. Vegetative umbilical lesions.
Figure 2 - Computed tomography of the abdomen indicates probable umbilical
endometriosis. Diagnosis of umbilical endometriosis on computed
tomography of the abdomen.
Figure 2 - Computed tomography of the abdomen indicates probable umbilical
endometriosis. Diagnosis of umbilical endometriosis on computed
tomography of the abdomen.
The biopsy of the primary lesion revealed the presence of skin associated with
stromal fragments and endometrial glands with no signs of malignancy. Umbilical
endometriosis was confirmed by analysis of clinical data, tomography scans, and
biopsy, and excision of the lesion with a safe margin was proposed (Figures 3a, b, and c). The biopsies were
subjected to histopathological examination. The final appearance after umbilical
reconstruction is shown in Figures 3d and
3e.
Figure 3 -
A: Initial surgical incision. Initial surgical incision
in the umbilical region; B: Excision of lesions with
free surgical margins. Excision of the lesions; C:
Umbilical tissue was excised and compared with the size of the
scalpel. Umbilical tissue was excised; D: Immediate
postoperative result of partial reconstruction of the umbilicus;
E: Late postoperative result of partial
reconstruction of the umbilicus.
Figure 3 -
A: Initial surgical incision. Initial surgical incision
in the umbilical region; B: Excision of lesions with
free surgical margins. Excision of the lesions; C:
Umbilical tissue was excised and compared with the size of the
scalpel. Umbilical tissue was excised; D: Immediate
postoperative result of partial reconstruction of the umbilicus;
E: Late postoperative result of partial
reconstruction of the umbilicus.
DISCUSSION
Cutaneous endometriosis is rare and is related to previous surgical procedures in
most cases. This condition is classified as primary when it occurs
spontaneously, as in the current case. The most common cutaneous manifestation
is umbilical, occurring in 34% of cases1,4,5.
Several hypotheses have been proposed to explain the pathophysiology of primary
umbilical endometriosis. The most accepted hypothesis in women with concomitant
pelvic endometriosis is embolization of endometrial tissue via hematogenous or
lymphatic routes. However, metaplasia of Müllerian duct remnants is more likely
in patients with cutaneous endometriosis and may be due to inflammatory,
hormonal, or traumatic causes2,4.
These lesions may present with specific symptoms, including a palpable
erythematous or violaceous nodule of a brownish color with increased volume, and
cyclical bleeding and pain during the menstrual period. These lesions may be
asymptomatic, in which case the diagnosis is more difficult2,4. In the
current case, the patient presented signs and symptoms consistent with previous
reports, including a brown and violaceous nodule associated with pain and
bleeding during the menstrual period.
The diagnosis is established by a compatible clinical picture and complementary
examinations. Ultrasound can be performed and reveals a cystic appearance.
Computed tomography and magnetic resonance imaging are better for delimiting the
lesion and making a differential diagnosis1,2.
Although several methods for diagnosis are available, biopsy is the most reliable
and useful. This histopathological examination reveals the presence of
endometrial tissue, with endometrial glands lined by pseudostratified columnar
epithelium, which may present active secretion or free erythrocytes.
Immunohistochemistry can be used to assess the presence of estrogen and
progesterone receptors, which further confirms the diagnosis2,4.
Differential diagnosis should be made for benign and malignant lesions, including
hernias, entrapment of ilioinguinal or iliohypogastric nerves, umbilical polyp,
melanocytic nevus, suture granuloma, hemangioma, omphalitis, melanoma,
adenocarcinoma, squamous cell carcinoma, and basal cell carcinoma1,2,5.
The treatment of choice is surgical, with complete excision of the lesion and
umbilical reconstruction if necessary. The nodule should be removed with safe
margins to prevent lesion recurrence. After the procedure, clinical treatment
with oral contraceptives or other hormonal agents may be implemented to prevent
recurrence1,2,4.
The evolution is benign in most cases. However, in a few cases, carcinomatous
transformation may occur, requiring frequent assessment4.
CONCLUSION
The described approach has an excellent prognosis and low risk of malignancy.
This favorable evolution depends on adequate management of the patient.
Moreover, accurate differential diagnosis (for benign or malignant lesions) is
essential to determine appropriate treatment.
COLLABORATIONS
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CBM
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Analysis and/or interpretation of data; final approval of the
manuscript; writing the manuscript or critical review of its
contents.
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AMG
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Analysis and/or interpretation of data; final approval of the
manuscript; writing the manuscript or critical review of its
contents.
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ACV
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Analysis and/or interpretation of data; final approval of the
manuscript; writing the manuscript or critical review of its
contents.
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ARM
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Analysis and/or interpretation of data; final approval of the
manuscript; writing the manuscript or critical review of its
contents.
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BAA
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Analysis and/or interpretation of data; final approval of the
manuscript; writing the manuscript or critical review of its
contents.
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ADBM
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Analysis and/or interpretation of data; final approval of the
manuscript; writing the manuscript or critical review of its
contents.
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ABFB
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Analysis and/or interpretation of data; final approval of the
manuscript; writing the manuscript or critical review of its
contents.
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AAR
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Analysis and/or interpretation of data; final approval of the
manuscript; writing the manuscript or critical review of its
contents.
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GNM
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Analysis and/or interpretation of data; final approval of the
manuscript; completion of surgeries and/or experiments; writing the
manuscript or critical review of its contents.
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REFERENCES
1. Federação Brasileira das Associações de Ginecologia e Obstetrícia
(FEBRASGO). Manual de endometriose. São Paulo: FEBRASGO; 2015.
2. Jaime TJ, Jaime TJ, Ormiga P, Leal F, Nogueira OM, Rodrigues N.
Endometriose umbilical: relato de um caso e seus achados dermatoscópicos. An
Bras Dermatol. 2013;88(1):121-4. DOI: http://dx.doi.org/10.1590/S0365-05962013000100019
3. Pramanik SR, Mondal S, Paul S, Joycerani D. Primary umbilical
endometriosis: A rarity. J Hum Reprod Sci. 2014;7(4):269-71. DOI: http://dx.doi.org/10.4103/0974-1208.147495
4. Fancellu A, Pinna A, Manca A, Capobianco G, Porcu A. Primary
umbilical endometriosis. Case report and discussion on management options. Int J
Surg Case Rep. 2013;4(12):1145-8. DOI: http://dx.doi.org/10.1016/j.ijscr.2013.11.001
5. Garcia AMC, Silveira PS Jr, Garcia BGBC, Assis MG. Endometriose
cutânea umbilical: Relato de caso e revisão da literatura. ACM Arq Catarin Med.
2009;38(Supl. 1):254-6.
1. Acadêmica em Medicina, Universidade de Franca,
Franca, SP, Brazil.
2. Sociedade Brasileira de Cirurgia Plástica, São
Paulo, SP, Brazil.
3. Associação Médica Brasileira, São Paulo, SP,
Brazil.
Corresponding author: Carla Batista
Moisés, Rua João da Silva Ranhel, 1850, Apt. 304, Bloco 1 - Nucleo Agricola
Alpha - Franca, SP, Brazil. Zip Code 14403-175. E-mail:
carlabmoises@live.com
Article received: November 23, 2017.
Article accepted: June 22, 2018.
Conflicts of interest: none.
