AJR 2002; 179:1480
© American Roentgen Ray Society
Radiologic—Pathologic Conference of Keller Army Community Hospital at West Point, the United States Military Academy
Calcified Lymphangioma of the Gonadal Vein
Liem T. Bui-Mansfield1,2,
Keith J. Kaplan3 and
Charles Hollcraft1
1 Department of Radiology, Keller Army Community Hospital, West Point, NY
10996-1197.
2 Department of Radiology, Wake Forest University School of Medicine, Medical
Center Blvd., Winston-Salem, NC 27157-1088.
3 Department of Pathology, Walter Reed Army Medical Center, 7100 Georgia Ave.
N.W., Washington, DC 20307-5001.
Received March 14, 2002;
accepted after revision April 3, 2002.
From the radiologic—pathologic correlation conferences of Keller Army
Community Hospital at West Point, the United States Military Academy.
The opinions and assertions contained herein are those of the authors and
should not be construed as offical or as representing the opinions of the
Department of the Army or the Department of Defense.
Address correspondence to L. T. Bui-Mansfield.
A 64-year-old woman presented with chronic right flank pain. Radiography of
the abdomen showed a calcified mass overlying the left psoas muscle. CT
revealed a 4.2 x 4.0 x 2.2 cm nonenhancing soft-tissue mass
containing phlebolithlike calcifications anterior to the left psoas muscle
(Fig. 1A). The mass displaced
the left ureter medially. Exploratory laparotomy
(Fig. 1B) showed that the mass
arose from the left gonadal vein. The histologic diagnosis was lymphangioma
(Figs. 1C and
1D).
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Fig. 1A. —64-year-old woman with calcified lymphangioma of left gonadal
vein. Contrast-enhanced CT scan shows nonenhancing soft-tissue mass
(arrow) with phlebolithlike calcifications anterior to left psoas
muscle and displacing left ureter medially.
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Fig. 1B. —64-year-old woman with calcified lymphangioma of left gonadal
vein. Photograph of surgical specimen consists of fairly well circumscribed
fatty-appearing mass with multiple hemorrhagic, compressible, spongelike
lesions (arrow). Vascular lumen is present.
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Fig. 1C. —64-year-old woman with calcified lymphangioma of left gonadal
vein. Photomicrograph of surgical specimen shows multiple cystic spaces (C)
lined by attenuated endothelium resembling normal lymphatics. Large vessel
with phlebolith (P) is present. (H and E, x4)
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Fig. 1D. —64-year-old woman with calcified lymphangioma of left gonadal
vein. Photomicrograph of surgical specimen shows cystic spaces (C) lined by
attenuated endothelium resembling normal lymphatics with dense lymphoid
aggregates (arrow) and engorged vascular spaces (V). (H and E,
x10)
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Malignant neoplasms (liposarcoma, leiomyosarcoma, malignant fibrous
histiocytoma, malignant peripheral nerve sheath tumor, fibrosarcoma, malignant
mesenchymoma, and rhabdomyosarcoma) are found in the retroperitoneum three
times more often than are benign neoplasms (schwannoma, fibromatosis,
hemangiopericytoma, angiomyolipoma, lipoma, and paraganglioma)
[1]. Lymphangioma of the
retroperitoneum is rare
[2,3,4].
Lymphangiomas are malformations arising from sequestrations of lymphatic
tissue that fail to communicate normally with the lymphatic system.
Traditionally, they were classified as simple, cavernous, or cystic
lymphangiomas. This classification has been replaced by the all-inclusive
"lymphangioma" because many of these entities have both cystic and
cavernous components, and a cystic lymphangioma is a long-standing cavernous
lymphangioma.
Lymphangiomas are well-circumscribed lesions composed of one or more large
interconnecting cysts (cystic lymphangioma) or ill-defined, spongelike
compressible lesions (cavernous lymphangioma). Lymphangiomas are equally
predominant in males and females. They appear by the end of the second year of
life in most patients (90%); lymphangiomas that manifest in adults are usually
cutaneous or intraabdominal
[2].
Intraabdominal lymphangiomas are rare, representing only 2% of all
lymphangiomas. Their most common locations, in descending order of frequency,
are the mesentery, omentum, mesocolon, and retroperitoneum (0.1% of all
lymphangiomas). Symptoms in the first three locations are a palpable mass and
pain due to intestinal obstruction, volvulus, and infarction. Retroperitoneal
lymphangiomas present much later in life than other intraabdominal
lymphangiomas, occurring as a large palpable mass that displaces adjacent
retroperitoneal organs [2].
On sonography, a lymphangioma appears as a cystic mass with septations or
echogenic debris. CT reveals a soft-tissue mass of multiple homogeneous
nonenhancing areas with variable attenuation values, depending on whether the
fluid is chylous or serous. Calcification is uncommon (10%) and is usually
mural [3]. Rarely, a calcified
retroperitoneal lymphangioma has phlebolithlike calcifications that may mimic
a hemangioma [4]. These
phlebolithlike structures are found in the engorged vascular spaces in the
lymphangioma (Fig. 1C).
Treatment for lymphangioma is complete surgical excision.
References
- Kransdorf MJ, Murphey MD. Imaging of soft tissue
tumors. Philadelphia: Saunders, 1997:3
-35
- Weiss SW, Goldblum JR. Enzinger and Weiss's soft tissue
tumors, 4th ed. St. Louis: Mosby, 2001:956
-967
- Davidson AJ, Hartman DS. Lymphangioma of the retroperitoneum: CT
and sonographic characteristics. Radiology
1990;175:507
-510[Abstract/Free Full Text]
- Hanelin LG, Schimmel DH. Lymphangioma of the pancreas exhibiting an
unusual pattern of calcification. Radiology
1977;122:636[Abstract]
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