Penile Lymphangioma: review of the literature with a case presentation

Background Penile lymphangiomas are rare manifestations of lymphangiomas or lymphatic malformations which are more commonly found in the head or neck region of the body. Lymphangiomas are further categorized as lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, or acquired lymphangiomas (also known as lymphangiectasia), based on their depth and etiology. Results A literature review revealed only 30 cases of penile lymphangioma between 1947 and March 30, 2018. Several causes were attributed to the acquired penile lymphangiomas, including trauma, phimosis, and infection. While penile lymphangiomas can be initially mistaken for an infection, a thorough history and physical examination is sufficient to clinically diagnose a lymphangioma of the penis. Historically, surgical excision has been the gold standard of treatment for this condition. When asymptomatic, patients may opt for conservative management with avoidance of mechanical trauma alone. Other physicians have revealed novel treatment plans to rid patients of their penile lymphangioma such as a staged laser procedure. Conclusion In this article, we elucidate the causes, symptoms, treatments, and outcomes associated with penile lymphangiomas found in the literature while also presenting the case of a 30-year-old African-American man diagnosed with acquired penile lymphangioma.


Background
Redenbacher et al. first described lymphangiomas, or "lymphatic malformations," in 1828 [1]. Since then, lymphangiomas have been classified further in the literature as lymphangioma circumscriptum, cavernous lymphangiomas, cystic hygromas, and acquired lymphangiomas (also known as lymphangiectasias). Lymphangiomas are most commonly found in the head and neck, and two-thirds of all lymphangiomas are found by two years of age. The literature provides detailed pathological, radiographical, and clinical findings associated with lymphangiomas of the head and neck. On the contrary, fewer than 50 cases of lymphangiomas of the penis have been reported since the first description by Ferris et al. in 1944. Presumably, lymphangiomas of the penis are under-reported because the penile lesions, unlike the pathological counterparts in the head/neck, often go unnoticed by not only the physician but also the patient [2]. Furthermore, lymphangiomas of the penis are often misdiagnosed and mistreated as genital warts, molluscum contagiosum or gonorrhea [3]. The objective of this study is to define the different types of penile lymphangiomas by etiology, clinical findings, and treatments. Also, we report a case of acquired lymphangioma of the penis in a 30-year-old male treated successfully by surgical excision.

Methods
We conducted a literature review identifying all publications using the keywords: "penile" OR "penis" AND "lymphangioma" OR "lymphangiectasis" OR "lymphangiectasia." Publications found in languages other than English were excluded. Publications reporting lymphangiomas of the scrotum, perineum, and/or surrounding area not including the penis were excluded. The publications reporting benign transient lymphangiectasis of the penis (BTLP) were also excluded as BTLP, commonly known as sclerosing lymphangitis, is a transient condition that often results after sexual activity and holds minimal medical or surgical relevance. Classifications of penile lymphangioma found in Table 1 corresponding to the case classification from their original publication include "Acquired" (lymphangioma), "Cavernous" (lymphangioma), (lymphangioma) "Circumscriptum," and (lymphangioma) "Circumscriptum Cysticum" (for cystic hygroma). The classification "Acquired [inferred]" was reserved for cases of lymphangiectasis/lymphangiectasia as well as reported cases of lymphangioma circumscriptum resulting from non-congenital causes. Although controversy exists in the difference between lymphangioma and lymphangiectasis/lymphangiectasia, all cases of lymphangiectasis/lymphangiectasia are reported as "Acquired [inferred]" in Table 1 for simplicity.

Case presentation
A 30-year-old African-American male presented to his primary care physician with a chief complaint of a several-year history of unhealing wounds on the right side of his penile shaft after his penis was caught in his zipper several years ago. Patient became concerned after noting white penile discharge 2 weeks prior. He denies any anal or oral lesions as well as exposure to any sexually transmitted diseases (STDs). Following outpatient specialty referral, the dermatologist reported excess skin tissue with underlying edema circumferentially on the distal penile shaft with overlying multiple firm skin-colored papules, some with exophytic crusting [Fig. 1]. Chlamydia trachomatis, human immunodeficiency virus (HIV), Neisseria gonorrhea and syphilis were negative. Subsequent biopsy found dilated vascular channels consistent with benign acquired lymphangioma of the penis (Fig. 2), and the patient was referred to urology for evaluation and management.
With the urologist, the patient elected for surgical intervention due to cosmetic concerns despite the asymptomatic nature of the lymphangioma. The patient underwent circumcision for redundant prepuce, excision of the skin lesion and penile foreskin reconstruction. A circumferential incision was made on the mucosa 0.5 cm proximal to the glans, distal to the lymphangioma. The foreskin was then retracted and another circumferential incision was made around the mucosal skin. The foreskin was then dissected using Bovie cautery and blunt dissection while the foreskin with lymphangioma tissue was excised. Intraoperative and postoperative courses were unremarkable. At 1-month follow-up, the patient reported no pain, erythema or discharge from the wound.

Results
The current literature review identified 27 cases in 25 publications. In the largest group, the 18 acquired  Multiple firm skin-colored papules, some with exophytic crusting and underlying edema, present on the right side of the patient's penile shaft, immediately proximal to the glans lymphangiomas of the penis identified in the literature review encompassed a wide variety of cases. One was attributed to phimosis and 2 to trauma [4,5]. Two iatrogenic cases resulted from correction of phimosis [6,7]. One infectious case was attributed to filariasis (1 case), and 1 inflammatory case was attributed to hidradenitis suppurativa (1 case) [8,9]. Two idiopathic cases were previously mistaken for genital warts and gonorrhea [10,11].
Lymphangioma circumscriptum of the penis in this literature review was reported in eight, five of which were ruled to be congenital. The other three were notable for comorbidities of ulcerative colitis, recurrent cellulitis, and Lichen planus [12][13][14]. The only case of lymphangioma circumscriptum cysticum or cystic hygroma was found to have a congenital origin [15]. Lastly, cases of cavernous lymphangioma were attributed to a tumor (2 cases) and one with an unknown cause [16,17].

Discussion
Lymphangiomas are generally described as uncommon, hamartomatous malformations of the lymphatic system. Based on depth and size of the lymph vessels, classifications include lymphangioma circumscriptum (most superficial), cavernous lymphangioma and cystic hygroma (most deep). Further subdivisions derive from perceived cause. Congenital lymphangiomas are thought to result from fetal lymph vessels that failed to involute and/or failed to join with the central lymphatic system. On the contrary, acquired lymphangiomas may result from trauma, certain infections (cellulitis, neoplastic disease, tuberculosis, filariasis), radiotherapy, pregnancy, scleroderma, severe phimosis or STDs [10].
Although the causes of these various types of lymphangioma may differ, the clinical presentation is often similar. The majority of the penile lymphangiomas present as asymptomatic, fluid-filled, translucent lesions or vesicles most commonly on the shaft or coronal sulcus of the penis. Symptomatic lesions, on the other hand, typically focus on sexual dysfunction. One case of acquired lymphangioma presented with severe phimosis and inability to produce an erection [4]. Also, one case of lymphangioma circumscriptum caused recurrent infection, intermittent drainage and sexual inactivity [18]. Given the similarities in clinical presentation, a proper diagnosis becomes contingent on a thorough history and physical examination in the case of lymphangioma of the penis. To that end, clinicians seeking to properly diagnose and treat lymphangiomas of the penis must effectively rule out certain infectious diseases such as molluscum contagiosum and gonorrhea [19]. This is generally possible following a thorough history and physical examination alone. However, in some rare cases, a biopsy with accompanying pathology report or an infectious disease workup may be necessary. Dermatology consultation may be selected for unclear cases.
Interestingly, Errichetti et al. recently elucidated the potential role of dermoscopy in the diagnosis of penile lymphangioma by describing the presence of "yellowish-reddish, well-demarcated, round or oval lacunae surrounded by whitish areas or lines," which may be common characteristics of lymphangiomas [4,20]. This observation warrants further investigation into the utility of dermoscopy as a quick, non-invasive method of definitively diagnosing lymphangiomas.
Many authors may argue that lymphangiomas of the penis do not always require treatment, given the mostly asymptomatic nature of these lesions. However, patients may request intervention for cosmetic reasons. Of the 18 acquired lymphangiomas reported in Table 1 (including the present case), 9 were treated by surgical excision [2,5,9,10,[21][22][23] only one of which recurred 11 months following surgery [24]. And, one case reported an electrofulguration (or electrocautery) of the visible papulo-vesicles on the penis [11]. Shi et al. found that the acquired lymphangioma of the penis was amenable to 2940-nm Erbium-doped Yttrium Aluminum Garnet laser once every 2-3 weeks, wherein the lesions disappeared after the fourth session with no evidence of recurrence, dyspigmentation or paresthesia [7]. Bardazzi et al. utilized high-frequency electric currents, called diathermy, to successfully obliterate the acquired lymphangioma without evidence of recurrence [25]. On the other hand, Zhang et al. advocated for a "watch and wait" policy that interestingly allowed the lesions to self-heal within three weeks time [6]. Lymphangiomas that develop following circumcision may spontaneously resolve. Abstinence from treatment was observed in two patients, one of whom opted for protecting the lesions from mechanical trauma and applying a silver sulfadiazine cream to any ruptured lesions [19,26]. This management decreased the number of existing lesions and prevented new lesions. Finally, one patient was waiting for surgery to correct phimosis; no follow-up information was provided [4]. Neither treatment nor follow-up was documented in the remaining two cases [8,11].
Of the 3 cavernous lymphangiomas reported in Table 1, one underwent circumcision with no sign of recurrence following the treatment [16]. The other two cases failed to specify treatment [17]. Of the 8 cases of lymphangioma circumscriptum of the penis, 3 underwent surgical resection successfully with no signs of recurrence [18,27,28]. Another patient with lymphangioma circumscriptum (Table 1) underwent 3 surgeries with recurrence every time. For the fourth operation the physicians adopted a more radical surgical approach, in which the penile shaft was denuded and buried in the scrotum [3]. Six months later, the penile shaft was raised, and the skin was reconstructed using a scrotal graft. The outcome of this procedure was favorable with the only noteworthy complication of growth of transposed hair, treated cosmetically. Of the remaining 4 lymphangioma circumscriptum cases shown in Table 1, treatment was denied for 1, and the remaining 3 had no information on treatment or outcome [12][13][14]29].

Conclusions
In summary, penile lymphangioma can be divided into four categories: 1) acquired, 2) lymphangioma circumscriptum, 3) cavernous lymphangioma, and 4) cystic hygroma. Commonly mistaken for infectious lesions, lymphangiomas underscore the importance of an appropriate history and physical to properly identify and treat the lymphatic malformation.

Funding
There are no funding sources to report.

Availability of data and materials
The datasets used and/or analyzed for this study can be procured from the corresponding author upon request.
Author's contributions MM assisted in manuscript composition and final review. SA assisted in manuscript composition and data collection. *MM and SA contributed equally to this submission. Hayan Jaratli provided the images and histology description. AD edited the manuscript and confirmed the accuracy of manuscript content. All authors read and approved the final manuscript Ethics approval and consent to participate This study was approved by the Henry Ford Hospital Institutional Review Board (IRB #11419).

Consent for publication
Upon outpatient follow-up, the patient consented to publishing his information and images (although they have been anonymized). The appropriate forms have been uploaded.