AJR 2001; 177:1090
© American Roentgen Ray Society
Radiologic-Pathologic Conferences of
The University of Texas
M. D. Anderson Cancer Center |
Cystic Lymphangioma of the Pancreas
Titus R. Koenig1,
Evelyn M. Loyer2,
Gary J. Whitman2,
A. Kevin Raymond3 and
Chusilp Charnsangavej2
1
Department of Radiology, The University of Texas—Houston Medical School,
6431 Fannin St., Houston, TX 77030.
2
Department of Radiology, The University of Texas M. D. Anderson Cancer Center,
P. O. Box 57, 1515 Holcombe Blvd., Houston, TX 77030.
3
Department of Pathology, The University of Texas M. D. Anderson Cancer Center,
Houston, TX 77030.
Received March 13, 2001;
accepted after revision April 4, 2001.
Address correspondence to G. J. Whitman.
Article
A 20-year-old man had a 3-day history of left-sided abdominal pain with
nausea and vomiting. A CT scan showed a 7-cm multiseptated cystic mass in the
lesser sac (Figs. 1A and
1B). The mass abutted the body
and tail of the pancreas, the posterior wall of the stomach, and the
transverse colon. Intraoperatively, a complex cystic mass was found in the
lesser sac, attached to the inferior border of the midbody of the pancreas.
The mass was excised, necessitating resection of a 1-cm portion of adherent
pancreas. Histologically, the cystic mass was composed of dilated
endothelia-lined cystlike spaces separated by fibrous stroma containing
abundant lymphocytes (Fig. 1C).
The final pathologic diagnosis was benign cystic lymphangioma.
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Fig. 1C. —20-year-old man with cystic lymphangioma of pancreas.
Photomicrograph of histologic specimen shows enlarged cystlike spaces
(arrow) lined by endothelial cells. Numerous lymphocytes are present
in fibrous stroma. (H and E, x40)
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Lymphangiomas are benign neoplasms arising from the lymphatic system.
Lymphangiomas are common in pediatric patients in the soft tissues of the neck
and the axilla. Abdominal lymphangiomas are rare, accounting for less than 1%
of all lymphangiomas. Lymphangiomas of the abdomen most commonly involve the
mesentery and the retroperitoneum
[1]. Pancreatic lymphangiomas
are extremely rare; only a few cases have been reported. The clinical
presentation is nonspecific and includes abdominal pain, nausea, vomiting, and
a palpable mass [2]. Symptoms
may develop acutely in children, whereas in adults, complaints tend to evolve
over months to years before diagnosis
[3]. Pancreatic lymphangiomas
are more common in women than in men, and these tumors have been reported in
all age groups [4].
Lymphangiomas of the pancreas may measure up to 20 cm in greatest dimension
[2].
Three histologic types of lymphangiomas have been described: cystic,
capillary, and cavernous. Only the cystic and cavernous types have been
reported in the pancreas [2].
Lymphangiomas are considered to be of pancreatic origin if they are in the
pancreatic parenchyma, adjacent to the pancreas, or connected to the organ by
a pedicle [2]. Macroscopically,
cystic lymphangiomas appear as soft, multiloculated cystic masses that contain
serous, serosanguinous, or lymphatic fluid. Histologically, dilated lymphatic
channels of varying size are seen, separated by thin septa. The cystic spaces
are lined with flattened or cuboidal endothelial cells. Aggregates of
lymphocytes are found in the lumina and the adjacent stroma. The septa and
walls contain occasional smooth muscle fascicles and various amounts of
collagenous connective tissue
[2].
CT usually shows well-circumscribed homogeneous cystic masses in or
adjacent to the pancreas. Multiple fine septations and thin walls may enhance
after IV contrast injection. Cystic lymphangiomas, if large enough, may exert
considerable mass effect on adjacent organs such as the stomach, spleen,
kidney, and liver. Rarely, phlebolithlike calcifications may be present.
Sonography usually shows an anechoic or hypoechoic fluid-filled, multiseptated
mass in the pancreatic region. Some hyperechoic masses have been reported
[4]. MR imaging may confirm the
sonographic and CT findings, but MR imaging usually does not provide much new
information. The cystic spaces appear hypointense on T1-weighted images and
hyperintense on T2-weighted images.
The differential diagnosis includes simple cysts, pseudocysts,
cystadenomas, and cystadenocarcinomas
[2]. CT and sonography usually
do not distinguish a lymphangioma from a pancreatic malignancy. Fine-needle
aspiration may suggest the diagnosis of pancreatic lymphangioma. The
definitive diagnosis of pancreatic lymphangioma, however, can be made only by
excision and histopathologic examination
[1,
4].
Pancreatic lymphangiomas are benign neoplasms, but they can be locally
invasive [3]. Surgical excision
is usually curative. Incomplete excision is likely to lead to a local relapse.
Partial pancreatectomy may be necessary in some cases
[3,
4].
References
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Abe H, Kubota K, Noie T, Bandai Y, Makuuchi M. Cystic lymphangioma
of the pancreas: a case report with special reference to embryological
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Paal E, Thompson LD, Heffess CS. A clinicopathologic and
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literature. Cancer
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de Perrot M, Rostan O, Morel P, Le Coultre C. Abdominal
lymphangioma in adults and children. Br J Surg
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Hayashi J, Yamashita Y, Kakegawa T, Ogata M, Nakashima O. A case of
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