Umbilical endosalpingiosis: a case report

Introduction: Endosalpingiosis describes the ectopic growth of Fallopian tube epithelium. Pathology confirms the presence of a tube-like epithelium containing three types of cells: ciliated, columnar cells; non-ciliated, columnar secretory mucous cells; and intercalary cells. We report the case of a woman with umbilical endosalpingiosis and examine the nature and characteristics of cutaneous endosalpingiosis by reviewing and combining the other four cases existing in the international literature. Case presentation: A 50-year-old Caucasian, Greek woman presented with a pale brown nodule in her umbilicus. The nodule was asymptomatic, with no cyclical discomfort or variation in size. Her personal medical, surgical and gynecologic history was uneventful. An excision within healthy margins was performed under local anesthesia. A cystic formation measuring 2.7×1.7×1 cm was removed. Histological examination confirmed umbilical endosalpingiosis.

Umbilical endosalpingiosis is a very rare manifestation of the non-neoplasmatic disorders of the Müllerian system. It appears with cyclic symptoms of pain and swelling of the umbilicus, but not always. The disease is diagnosed using pathologic findings and surgical excision is the definitive treatment.

Endosalpingiosis is a rare clinical entity that describes the ectopic growth of Fallopian tube epithelium [1]. Endosalpingiosis, endometriosis and endocervicosis con- stitute the triad of non-neoplastic disorders of the Mül- lerian system. These pathologies are found in isolation, but are more commonly found in association with one another [2,3]. The diagnosis of these pathologies is made histologically. In the case of endosalpingiosis, pathology confirms the presence of a tube-like epithe- lium containing three types o f cells: ciliated, columnar cells; non-ciliated, columnar mucous secretor cells; and the so-called intercalary or peg cells [4,5]. We report the case of an adult woman with umbilical endosalpingiosis and elucidate the nature and character- istics of cutaneous endosal pingiosis by reviewing and combining the four cases existing in the international literature. Case presentation A 50-year-old Caucasian, Greek woman presented with a pale brown nodule in her umbilicus. The nodule was asymptomatic, with no cyclical discomfort or variation in size. Her personal medical, surgical and gynecologic history was uneventful. An excision within healthy mar- gins was performed under local anesthesia. A cystic for- mation measuring 2.7×1.7 ×1 cm was removed. There were no signs of malignancy and no evidence of endo- metrial component in her surrounding tissue. Pathologic examination showed a unilocular cyst with papillary projections into the lumen (Figures 1 and 2). The cyst was surrounded by edematous fibrous tissue. The lining consisted of epithelium cells, both cuboidal and ciliate (Figure 3). Immunohistochemical staining showed posi- tivity for keratins AE1/AE3 (Figure 4). Finally, a histolo- gical examination posed the diagnosis of cutaneous endosalpingiosis.

The term endosalpingiosis was employed for the first time by Sampson et al . in 1930. Under that term, the author designated any unusual growth and invasion of * Correspondence: [email protected] 1Department of Surgery, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece Full list of author information is available at the end of the article Papavramidis et al . Journal of Medical Case Reports 2010, 4:287 http://www.jmedicalcasereports.com/content/4/1/287 JOURNAL OF MEDICAL CASE REPORTS © 2010 Papavramidis et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. tubal epithelium in tubal stumps, in subjects who had undergone previous salpingec tomy or tubal sterilization [1]. The different theories for the pathogenesis of endo- salpingiosis are similar to those for endometriosis, since those two entities, together w ith endocervicosis, consti- tute the non-neoplastic disorders of the Müllerian sys- tem. The different models can be traced back to two basic ideas. One group of theories is based on the fact that endometrial cells (or their precursors) are trans- ported by various routes (transtubal, hematogenous, lymphogenous or by direct apposition) and implanted in the affected organ. The other group of theories suggests that Müllerian ectopias are the result of metaplastic pro- cesses in the target organ (co elomic metaplasia theory, secondary Müllerian system) or from scattered embryo- nic rest [6-8]. We believe that the first group of theories is inadequate when explaining the origin of endosalpin- giosis in our case report, since she has a free gynecolo- gic, obstetric and surgical hi story. In our case report, it is likely that the Müllerian ectopia resulted either from metaplastic processed or scattered embryonic rest. Non-neoplastic glandular proliferation showing spon- taneous Müllerian differentiation has been described in many sites including the vagina [9], uterine cervix [10], urinary bladder [11,12], appendix [13], peritoneum [14,15], abdominal wall (in guinal channel, umbilicus) [2,16], and the lymph nodes [17]. However, to the best of our knowledge, this is the first case of a patient with spontaneous endosalpingiosis presenting as a nodule on the abdominal wall. The differential diagnosis of nodular umbilical lesions should include a wide range of diseases; such as hernia, keloid, abscess, lipoma, hematoma, sebaceous cyst, ade- nocarcinoma [primary of metastatic (Sister Joseph nodule)], melanoma, suture granuloma, pyogenic granu- loma, desmoid tumor, sarcoma, lymphoma, endometrio- sis, and endosalpingiosis. Of course, the final diagnosis should be made by pathology [18]. In the international literature, this is the fifth case of umbilical endosalpingiosis. The other four cases refer to patients with previous medical history of gynecologic procedures [5,19,20], while this is the first case of spon- taneous appearance. These lesions appear as nodules of the umbilicus and are usually brownish in colour. The main symptoms (besides the es thetic) are pain and size fluctuation with menstruat ion. The treatment of choice is surgical excision. Our opinion is that excision has to be made under local anesthesia, in order to minimize morbidity and hospitalization. However, the patient has to be notified that in the case of a reappearance of Figure 1 Unilocular cyst with papillary projections into the lumen (Hematoxylin and Eosin ×4) . Figure 2 Unilocular cyst with papillary projections into the lumen (Hematoxylin and Eosin ×10) . Papavramidis et al . Journal of Medical Case Reports 2010, 4:287 http://www.jmedicalcasereports.com/content/4/1/287 Page 2 of 4 abdominal pain (especially in the lower quadrant), a laparoscopy should be performed in order to exclude abdominal endometriosis.

Umbilical endosalpingiosis is a very rare manifestation of the non-neoplasmatic disorders of the Müllerian sys- tem. Normally, it appears with cyclic symptoms of pain and swelling of the umbilicus, but not always. The dis- ease is diagnosed using pathologic findings and surgical excision is the definitive treatment. Consent Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests The authors declare that they have no competing interests. Authors’ contributions TSP analyzed and interpreted the patient data and drafted the manuscript. KS received the patient in our out-patient department and was the principal surgeon. NM received the patient in our out-patient department, was the auxillary surgeon and drafted the manuscript. GK performed the pathological examination and was a major contributor in writing the manuscript. AC performed the pathological examination. STP was responsible for the overall treatment of the patient and corrected the manuscript. All authors read and approved the final manuscript. Author details 1Department of Surgery, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece. 2Department of Pathology, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece. Received: 23 October 2009 Accepted: 24 August 2010 Published: 24 August 2010

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