Rare Case of Ovarian Cystic Lymphangioma Managed with Laparoscopy

Introduction

Lymphangiomas are rare tumors of the lymphatic system comprised of multiple cystic spaces lined by endothelium. They are generally benign tumors. Lymphangiomas can be classified as capillary, cystic, or cavernous. They contain serous or chylous fluid. ¹ Lymphangiomas may arise anywhere in the body. In children, they more commonly arise in the head, neck, and axilla. In adults, most often they are superficial cutaneous or intraabdominal. ²

Lymphangioma of the ovary is rare. We identified only a few reports in a literature survey (PubMed).^3–10 Typically, lymphangiomas are slow-growing tumors. They may vary in their clinical presentation, and patients may remain asymptomatic for a long time. The tumors are most often discovered incidentally during abdominal or pelvic imaging studies or at surgery or autopsy.^8,9 Occasionally, they grow large and cause mass effect and compression of adjacent organs.^8,9 We present a case report of a cystic lymphangioma arising from the ovary in a 72-year-old postmenopausal woman which was successfully removed by laparoscopy.

Case

A postmenopausal 72-year-old woman was referred to a gynecologic laparoscopic surgeon for a consultation after an ultrasonography and CT scan found a right ovarian complex cyst measuring 6.3×4.4×3.2 cm. Initially, the patient had lower abdominal pain, bloating, constipation, and urinary frequency. She was hypertensive and was taking regular antihypertensive drugs. Pelvic examination revealed a mildly enlarged, nontender, mobile right adnexal mass. The remainder of the physical examination yielded unremarkable findings. The serum cancer antigen 125 (CA-125) concentration was within normal limits at 9.7 U/mL.

A total laparoscopic hysterectomy with bilateral salpingo-oophorectomy was planned, and an exploratory laparoscopy was performed. A 10-mm trocar was placed in the umbilicus, and three 5-mm secondary trocars were placed under direct visualization, suprapubically and in the left and right lateral position respectively. Exploration revealed an enlarged right ovary with a cystic mass, with no excrescences or implantation on the peritoneum (Fig. 1). The uterus was small in size, and a small amount of chylous milky ascites was observed in the pelvis. Peritoneal fluid was obtained for cytologic analysis. Both sides of the infundibulopelvic and tubo-ovarian ligaments were coagulated and cut, and a bilateral salpingo-oophorectomy was performed. The adnexa were removed intact at colpotomy using an Endobag™, Endo Catch Gold, Autosuture, Tyco Healthcare, Norwalk, USA. Multiple peritoneal biopsies were taken from the pelvic peritoneum, the peritoneum overlying the right and left paracolic gutter, and the subdiaphragmatic peritoneum. Frozen section of the right ovarian mass showed a gray and white multiple-lobulated mass containing multiple cystic areas filled with milky fluid. The diagnosis was benign mesenchymal mass with marked myxoid degeneration. The surgery was completed, assuring complete hemostasis and suturing of colpotomy laparoscopically. Final histologic analysis confirmed the diagnosis of cystic lymphangioma of the right ovary.

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FIG. 1.

Enlarged right ovary without any excrescences or implantation on the peritoneum.

Cytologic analysis of pelvic washings was significant for mesothelial cells, histiocytes, and lymphocytes without evidence of malignancy. The postoperative course was unremarkable.

Discussion

Lymphangiomas are rare, usually benign lesions of the lymphatic system. ¹ It is still uncertain whether they represent true neoplasms, hamartomas, or lymphangiectasis. ¹⁰ Their exact etiology and true incidence is unknown. In children, they more commonly arise in the head, neck, and axilla where the lymphatic sacs fail to communicate with the draining lymphatic channels.^10,11 In adults, their etiology is less understood. Some author suggest that their origin is by proliferation of lymphoid nests after inflammation, fibrosis, or genetic predisposition,^1,8,9,12 while many believe they are the end result of mechanical pressure, trauma, degeneration of lymph nodes, and disorders of the lymphatic vasculature.^8,9,12–14 Most often are superficial cutaneous or intraabdominal. ²

Suspicion of malignancy in our case was high because of the patient's age (72 years) and postmenopausal state, and because of the complexity of the ovarian mass on ultrasonography and CT scan, although her CA-125 was within normal limits. The cause of the rare ovarian lymphangioma in our case is not known. There was no history of precipitating trauma, infection, or exposure to radiation, and no previous surgery or adhesions. The lack of evidence of a reactive process suggests a neoplastic cause.

A few case reports of malignant lymphangiomas are available for review. Rice et al. ¹⁵ reported a histologically benign lymphangioma that was successfully resected at laparotomy, but which presented with contralateral ovarian involvement, liver metastasis, and diffuse intraperitoneal dissemination 6 months following surgery. The autopsy confirmed malignancy. ¹⁵ Another case report by Aristizabal et al. ⁷ described recurrence of a benign-appearing lymphangioma diffusely in the peritoneal cavity within 2 years of the open resection. These recurrences had the same benign appearance as the original lesion. Radiation therapy was required to control the disease. ⁷

These reports suggest that the histologic appearance of ovarian lymphangiomas may not reliably predict their subsequent clinical behavior. They also emphasize the need for complete wide excision with clean margins and for prolonged follow-up for at least 2 years. ⁷ Patients should undergo a pelvic examination and gynecologic sonography every 3 months.

As with other adnexal masses, pelvic ultrasonography is the preferred imaging method. Computed tomography (CT) or magnetic resonance imaging (MRI) may add important preoperative information about anatomical relationships with other structures, differentiate benign tumors from malignant tumors, and chylous fluid from blood and pus.^6,9

Laparotomy or laparoscopy are both acceptable routes of surgery for treatment. There are several reports in the literature of successful open resection of intraabdominal lymphangiomas, but only a few such reports describing the laparoscopic treatment of an ovarian lymphangioma.^4,5 The prognosis with laparoscopic treatment is usually excellent.^14,15 It has inherent advantages in the form of less intraoperative blood loss, early recovery, less morbidity, and low complication rate compared with laparotomy. In our case, the choice of laparoscopic surgery enabled us to reach the correct diagnosis and offer the right treatment, adhering to proper oncologic principles, with minimal morbidity. With advances in equipment, instruments, techniques, and training, the endoscopic approach to gynecologic surgery is a becoming more and more common, safe, and reliable procedure.

Conclusion

In conclusion, the present case illustrates that lymphangiomas should be included in the differential diagnosis of ovarian cystic masses. Even though they are being benign in nature the majority of the time, wide excision with clear margin and regular follow-up is mandatory. Laparoscopic excision is a safe and reliable approach for treatment.