Presacral lymphangioma: A rare cystic tumor

Jeffrey B. Albright, MD

Staff Colorectal Surgeon

Kaiser Permanente

Southern California Permanente Medical Group

Department of Surgery

South Bay Medical Center

Harbor City, CA

G. Peter Fakhre, MD

General Surgery Resident

Department of Surgery

Mayo Clinic Jacksonville

Jacksonville, FL

Kyle K. Shaddix, MD

General Surgery Resident

Department of Surgery

Mayo Clinic Jacksonville

Jacksonville, FL

Philip P. Metzger, MD

Assistant Professor of Surgery

Section of Colon & Rectal Surgery

Department of Surgery

Mayo Clinic Jacksonville

Jacksonville, FL

ABSTRACT

Introduction: Lymphangiomas are uncommon lesions that are typically found in the neck during childhood. Presacral lymphangiomas are extremely rare, and only one case has been reported previously in the literature.

Results and discussion: The authors report a case of presacral lymphangioma in an elderly man whose tumor was discovered during an evaluation for bilateral pulmonary emboli. The patient underwent resection of the lesion using a posterior extrasphincteric paracoccygeal approach. The lesion was excised in its entirety without any complications or damage to the rectal wall. The authors discuss the symptoms and complications, classification, differential diagnosis, and treatment of lymphangiomas.

Conclusion: Understanding the nature of lymphangiomas is essential to ensure that a proper operative approach is applied.

Presacral (ie, retrorectal) tumors are rare and can arise from various tissues in the pelvis. The most common complications associated with these lesions are bleeding, infection, and malignant degeneration. Treatment of presacral tumors consists of complete surgical excision; the operative approach depends on the size and location of the tumor and whether there is evidence of invasion. We report the case of a presacral lymphangioma that was discovered in an asymptomatic patient who had no history of pro-static or anorectal surgery. This is the second case of a presacral lymphangioma reported in the English-language literature.

CASE REPORT

Figure 1—Sagittal MRI showing the retrorectal cystic lesion. Note the margin between the lesion (arrow) and the distal sacrum and coccyx (single arrowhead) and the relationship to the rectum (double arrowhead).

A 69-year-old man was found to have a presacral mass during an evaluation for bilateral pulmonary emboli. Computed tomography (CT) scanning of the patient's abdomen and pelvis, performed to visualize the pelvic veins, demonstrated a 3.5-cm cystic mass adjacent to the left wall of the rectum. The patient was asymptomatic and had no history of anorectal or prostatic surgery. Digital rectal examination showed a subtle extra-luminal mass with a palpable cephalad margin. Results of the proctoscopic examination were normal. Magnetic resonance imaging (MRI) of the pelvis demonstrated a unilocular, thin-walled, 3.5 x 3.1-cm cyst posterior and to the left of the rectum, with some mass effect on the adjacent rectal wall (Figure 1). The signal intensity was consistent with a simple fluid collection. Fat planes were noted between the cyst and all other structures.

The patient underwent resection of the cystic lesion using a posterior extrasphincteric paracoccygeal approach. Because of the lesion's relatively small size, it was identified using bimanual palpation through the rectum. The tumor was located at approximately the S2-S3 level within the mesorectum, in close proximity to the rectal adventitia. The tumor was excised in its entirety without injury to the rectal wall.

Results of a frozen-section pathologic evaluation were indeterminate. Permanent section showed a fibrous wall containing chronic inflammation and foci of lymphoid tissue, with no definitive epithelial or mesothelial lining (Figure 2). These findings were consistent with a lymphangioma. The patient was discharged from the hospital on postoperative day 1 and had an uneventful recovery.

DISCUSSION

Lymphangiomas are uncommon lesions that are most often discovered during childhood. Approximately 75% of lymphangiomas occur in the neck and are referred to as cystic hygromas. Lymphangiomas of the axilla account for 20%, and the remaining 5% of lesions arise in the retroperitoneum and the thoracic and intra-abdominal soft tissues, including the mesentery of the small and large intestine and submucosa of the colon.^1-8 Presacral lymphangiomas are exceedingly rare, with only one other case reported in the English-language literature.⁹

Presacral tumors, including cystic lesions, tend to be asymptomatic. They occur more often in women and individuals between the ages of 40 and 60 years.¹⁰ The lesions are usually discovered incidentally during radiological examinations for other conditions.^10,11

The radiographic modalities commonly used to evaluate presacral tumors include CT scanning, MRI, and ultrasonography or endorectal ultrasonography.^11,12 The characteristics of these lesions on CT scanning, MRI, and endorectal ultrasonography are reviewed in the Table.^1,5,8,13-16

Symptoms and complications of presacral lesions

The symptoms of an intra-abdominal lymphan-gioma depend on its location and can include pain, a sensation of heaviness, fever, fatigue, weight loss, and obstructive symptoms secondary to extrinsic compression.¹ A submucosal lymphangioma may lead to microscopically heme-positive stools, hematochezia, or intussusception.^7,8,17 Asymptomatic lesions are often discovered incidentally.¹

Complications of presacral tumors include bleeding, infection, and malignant degeneration.¹⁸ Infections are rare and may result from manipulation or attempted aspiration of the lesion.

Classification

Presacral tumors can arise from several different tissues in the pelvis. They are commonly classified as congenital, neurogenic, osseous, inflammatory, or miscellaneous.^9,16 The three main classifications for lymphangiomas are simple (unicameral) cysts, cavernous (microscopic) cysts, and cystic (macroscopic) lesions.^13,19 Microscopic evaluation of well-circumscribed cystic lesions may identify an endothelial lin-ing.¹⁹ Their stroma creates a meshwork of collagen and fibrous tissue, and the wall contains focal aggregates of lymphoid tissue.¹⁹

Differential diagnosis

Practice

Points

• Presacral tumors are often discovered incidentally during radiological examinations.
• Symptoms of an intra-abdominal lymphangioma may include pain, sensation of heaviness, fever, fatigue, weight loss, and obstructive symptoms due to tumor burden.
• The radiologic and clinical characteristics of a lymphangioma are similar to those of other simple cystic presacral masses.
• Presacral tumors should be surgically excised to avoid complications such as bleeding, infection, and malignant degeneration.

The differential diagnosis of cystic presacral tumors encompasses various congenital lesions and other types of lesions.¹² Congenital cystic lesions include dermoid cysts, epidermoid cysts, tailgut cysts, duplication cysts, cystic forms of teratomas, and anterior sacral meningocele.^9,18 In rare cases, cystic lesions may represent cystic degeneration of a solid tumor or primary development of a malignancy.^11,12,20,21 Other uncommon lesions include subperitoneal adenomuci-nosis, pyogenic abscess, and sacral cystic lesions with retrorectal extension.¹⁸

Treatment

Presacral tumors should be surgically excised. The operative approach depends on the tumor's size and location and whether there is evidence of invasion.

Benign-appearing, low-lying S2 lesions with a diameter smaller than 4 to 6 cm are often amenable to posterior resection.^17,22,23 Tumors that are larger, located higher up, or suggestive of malignancy may require abdominal or combined abdomino-sacral approaches.¹¹ Lesions surrounded by inflamed tissue or associated with episodes of infection are best approached anteriorly or through a combined approach because of the potential for hemor-rhage.¹⁰ Recent reports describe successful anterior laparoscopic approaches for excising solid presacral tumors.^24,25

CONCLUSION

Intra-abdominal lymphangioma is an uncommon clinical entity that is known to occur in various anatomic locations. Presacral tumors are a heterogeneous group of lesions that arise from numerous cell types. Symptoms are not always present and depend on the lesion's characteristics, including its size and location. The radiologic and clinical characteristics of a lymphangioma are similar to those of other simple cystic presacral masses. Determining the nature of a presacral tumor or intra-abdominal lymphangioma is essential to ensure excision is undertaken using a proper operative approach.

Our patient's presacral lymphangioma had a classic presentation: the patient was asymptomatic and his lesion was found incidentally during abdominal imaging for an unrelated condition. The radiographic characteristics of the patient's lymphangioma were consistent with a benign, cystic, congenital pre-sacral mass, which was presumed to be a tailgut, dermoid, or epidermoid cyst. Because of the lesion's small size, the lack of invasion or involvement of surrounding structures, and its benign appearance and low-lying position, we elected to use a posterior approach for resection.

Radiation modality

Benefits of modality

Appearance of lesion

CT scanning

1. Usually most frequent initial screening examination.
2. Provides overall larger look at surrounding organs and structures, allowing for a more comprehensive examination.

Separated, thin-walled, fluid-filled cystic mass with low attenuation.

MRI

1. More detail of lesion?s location and it sassociation with surrounding structures.
2. Allows better assessment of the lesion?s location with regard to the sacral segments to aid in determining the optimal approach for surgical excision.

Fat content within the cyst helps narrow differential diagnosis to dermoid cyst, cystic lymphangioma, or lymphocele.

Endorectal ultrasonography

Clearly shows relationship of cystic lesion to layers of the rectal wall, which helps differentiate a duplication cyst (arising from submucosal layer of the rectal wall) from other cystic lesions.

Sharply defined cystic or multicystic mass demonstrating internal septations.

DISCLOSURE

The authors have no relationship with any commercial entity that might represent a conflict of interest with the content of this article and attest that the data meet the requirements for informed consent and for the Institutional Review Boards.

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