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63
Case
Langerhans Cell Histiocytosis Imaging Findings D. Randall
Report
of the Liver:
Radin1
The term Langerhans celihistiocytosis refers to proliferation of a unique histiocyte, the Langerhans cell [1 2]. According to the Writing Group of the Histiocyte Society [1], this term replaces the terms histiocytosis X, eosinophilic granuloma, Hand-Sch#{252}ller-Christian disease, and Letterer-Siwe disease. Clinical manifestations of Langerhans cell histiocytosis are varied and depend on the sites and extent of involvement. Common sites of involvement include bone marrow, lung, skin, liver, spleen, and lymph nodes. More than 90% of patients with disseminated disease are less than 21 years old
The hypoattenuating hepatic masses were moderately hyperintense on Ti -weighted MR images (Fig. 1C) but only slightly hyperintense on T2-weighted images (Fig. 1 D). The isoattenuating, exophytic
[2]. Sonographic,
hypotension, hypernatremia, and obtundation developed. MR imaging of the brain showed an enhancing 8-mm mass in the tuber cinereum. Results of endocrinologic evaluation indicated a diagnosis of panhypopituitarism including diabetes insipidus. Information obtained at that time indicated that the patient had had polyuria and polydipsia for 2 years. The results of a wedge biopsy of the liver were consistent with Langerhans cell histiocytosis. Hormonal replacement therapy was begun. Sonography of the liver 8 months after the first examination
,
CT, and MR findings
gerhans cell histiocytosis this report.
in an adult with Lan-
of the liver constitute
the subject
of
Case Report A 48-year-old woman had weakness, 7-kg weight loss, and intermittent abdominal pain, nausea, and diarrhea. Treatment for hypothyroidism was begun 6 weeks earlier. Serum level of bilirubin was elevated at 3.4 mg/dl (58 zmol/l; normal, 0.1 -1 .2 mg/dl [2-20 zmol/ I]) and serum level of alkaline phosphatase was elevated at 1074 U/I (normal, 20-90 U/I). Sonography showed a large irregular area of increased echogenicity centrally within the liver (Fig. 1A) and a 3-cm round, well-circumscribed, homogeneous, hyperechoic lesion with through-transmission Unenhanced CT showed
enhanced
posteriorly in the right lobe. a large irregular hypoattenuating
centrally in the liver (Fig. 1 B); attenuation -1 4 H. Several small hypoattenuating
of the
lesion
was
lesion as low
as
lesions were seen superiorly
and posteriorly in the right lobe. A large, mostly isoattenuating, exophytic mass extended medially from the inferior aspect of the right lobe. A similar exophytic mass extended posteriorly from the lateralsegment of the leftlobe. Received 1
Department
November
12, 1 991 ; accepted
of Radiology,
University
after
revision
of Southern
December
California
right-sided
showed
July 1992 0361-803X/92/1
591-0063
was
slightly
hyperintense
on Ti -weighted
no change in the large, irregular,
hyperechoic
hepatic lesion.
In the present case and two previous cases [3, 4] of Langerhans cell histiocytosis involving the liver in adults, sonography showed hyperechoic irregular and nodular lesions. In the present case and three previous cases [2-4], CT showed hypoattenuating irregular and nodular lesions. Attenuation of the lesions was consistent with fat in two cases. In the present case, the MR finding of hyperintense hepatic lesions on Ti -weighted images, with lower signal
30, 1991.
School
0 American
mass
Discussion
of Medicine, Los Angeles County-USC
90033-1084. AJR 159:63-64,
hepatic
images (Fig. 1E) and isointense on T2-weighted images. Although vessels were seen coursing through the masses, evidence of mass effect was present (Fig. 1C). A 3-cm mass located posteriorly in the right lobe of the liver, corresponding to the lesion shown by sonography, was hypointense on Ti -weighted images and hyperintense on T2-weighted images (Figs. 1C and 1D). Open liver biopsy was performed. Several hours after surgery,
Roentgen
Ray Society
Medical Center, 1200 N. State St. , Los Angeles, CA
Downloaded from www.ajronline.org by 190.102.31.167 on 10/18/15 from IP address 190.102.31.167. Copyright ARRS. For personal use only; all rights reserved
AJR:159, July 1992
RADIN
64
Fig. 1.-Langerhans cell histiocytosis In a 48-year-old woman.
of liver
A, Longitudinalsonogram shows a large, wellcircumscribed, hyperechoic mass with irregular margins (arrows) in liver. K = right kidney. B, Unenhanced CT scan shows a large, Irregular, hypoattenuating hepatic mass. C, Transverse Ti-weighted MR image shows a large, Irregular, hyperintense hepatic mass. Compression of portal vein (solid black arrow) and Inferior vena cava (open arrow) is seen. Hypointense mass (curved white arrow) is present in posterior right lobe. 0, Transverse T2-weighted MR Image shows large hepatic mass Is only slightly hyperintense. Smaller hepatic mass in posterior right lobe Is markedly hyperintense, consistent with cavernous hemangloma. E, Transverse Ti-weighted MR image shows slightly hyperintense exophytic mass (arrows) extending medially from right hepatic lobe.
intensity on T2-weighted fatty infiltration. However,
images, also was consistent the degree of mass effect
with focal fatty infiltration or fat-containing tumor, the diagnosis of Langerhans cell histiocytosis should be considered, especially in the presence of typical cutaneous, osseous, or pulmonary lesions or evidence of diabetes insipidus or pituitary insufficiency.
with in this
case would be exceptional for focal fatty change. Hepatic masses that may have sonographic, CT, and MR findings consistent
with
fat include
lipomas,
angiomyolipomas,
liver
cell adenomas, myelolipomas, and hepatocellular carcinomas with fatty metamorphosis [5]. In addition, some small (
63
Case
Langerhans Cell Histiocytosis Imaging Findings D. Randall
Report
of the Liver:
Radin1
The term Langerhans celihistiocytosis refers to proliferation of a unique histiocyte, the Langerhans cell [1 2]. According to the Writing Group of the Histiocyte Society [1], this term replaces the terms histiocytosis X, eosinophilic granuloma, Hand-Sch#{252}ller-Christian disease, and Letterer-Siwe disease. Clinical manifestations of Langerhans cell histiocytosis are varied and depend on the sites and extent of involvement. Common sites of involvement include bone marrow, lung, skin, liver, spleen, and lymph nodes. More than 90% of patients with disseminated disease are less than 21 years old
The hypoattenuating hepatic masses were moderately hyperintense on Ti -weighted MR images (Fig. 1C) but only slightly hyperintense on T2-weighted images (Fig. 1 D). The isoattenuating, exophytic
[2]. Sonographic,
hypotension, hypernatremia, and obtundation developed. MR imaging of the brain showed an enhancing 8-mm mass in the tuber cinereum. Results of endocrinologic evaluation indicated a diagnosis of panhypopituitarism including diabetes insipidus. Information obtained at that time indicated that the patient had had polyuria and polydipsia for 2 years. The results of a wedge biopsy of the liver were consistent with Langerhans cell histiocytosis. Hormonal replacement therapy was begun. Sonography of the liver 8 months after the first examination
,
CT, and MR findings
gerhans cell histiocytosis this report.
in an adult with Lan-
of the liver constitute
the subject
of
Case Report A 48-year-old woman had weakness, 7-kg weight loss, and intermittent abdominal pain, nausea, and diarrhea. Treatment for hypothyroidism was begun 6 weeks earlier. Serum level of bilirubin was elevated at 3.4 mg/dl (58 zmol/l; normal, 0.1 -1 .2 mg/dl [2-20 zmol/ I]) and serum level of alkaline phosphatase was elevated at 1074 U/I (normal, 20-90 U/I). Sonography showed a large irregular area of increased echogenicity centrally within the liver (Fig. 1A) and a 3-cm round, well-circumscribed, homogeneous, hyperechoic lesion with through-transmission Unenhanced CT showed
enhanced
posteriorly in the right lobe. a large irregular hypoattenuating
centrally in the liver (Fig. 1 B); attenuation -1 4 H. Several small hypoattenuating
of the
lesion
was
lesion as low
as
lesions were seen superiorly
and posteriorly in the right lobe. A large, mostly isoattenuating, exophytic mass extended medially from the inferior aspect of the right lobe. A similar exophytic mass extended posteriorly from the lateralsegment of the leftlobe. Received 1
Department
November
12, 1 991 ; accepted
of Radiology,
University
after
revision
of Southern
December
California
right-sided
showed
July 1992 0361-803X/92/1
591-0063
was
slightly
hyperintense
on Ti -weighted
no change in the large, irregular,
hyperechoic
hepatic lesion.
In the present case and two previous cases [3, 4] of Langerhans cell histiocytosis involving the liver in adults, sonography showed hyperechoic irregular and nodular lesions. In the present case and three previous cases [2-4], CT showed hypoattenuating irregular and nodular lesions. Attenuation of the lesions was consistent with fat in two cases. In the present case, the MR finding of hyperintense hepatic lesions on Ti -weighted images, with lower signal
30, 1991.
School
0 American
mass
Discussion
of Medicine, Los Angeles County-USC
90033-1084. AJR 159:63-64,
hepatic
images (Fig. 1E) and isointense on T2-weighted images. Although vessels were seen coursing through the masses, evidence of mass effect was present (Fig. 1C). A 3-cm mass located posteriorly in the right lobe of the liver, corresponding to the lesion shown by sonography, was hypointense on Ti -weighted images and hyperintense on T2-weighted images (Figs. 1C and 1D). Open liver biopsy was performed. Several hours after surgery,
Roentgen
Ray Society
Medical Center, 1200 N. State St. , Los Angeles, CA
Downloaded from www.ajronline.org by 190.102.31.167 on 10/18/15 from IP address 190.102.31.167. Copyright ARRS. For personal use only; all rights reserved
AJR:159, July 1992
RADIN
64
Fig. 1.-Langerhans cell histiocytosis In a 48-year-old woman.
of liver
A, Longitudinalsonogram shows a large, wellcircumscribed, hyperechoic mass with irregular margins (arrows) in liver. K = right kidney. B, Unenhanced CT scan shows a large, Irregular, hypoattenuating hepatic mass. C, Transverse Ti-weighted MR image shows a large, Irregular, hyperintense hepatic mass. Compression of portal vein (solid black arrow) and Inferior vena cava (open arrow) is seen. Hypointense mass (curved white arrow) is present in posterior right lobe. 0, Transverse T2-weighted MR Image shows large hepatic mass Is only slightly hyperintense. Smaller hepatic mass in posterior right lobe Is markedly hyperintense, consistent with cavernous hemangloma. E, Transverse Ti-weighted MR image shows slightly hyperintense exophytic mass (arrows) extending medially from right hepatic lobe.
intensity on T2-weighted fatty infiltration. However,
images, also was consistent the degree of mass effect
with focal fatty infiltration or fat-containing tumor, the diagnosis of Langerhans cell histiocytosis should be considered, especially in the presence of typical cutaneous, osseous, or pulmonary lesions or evidence of diabetes insipidus or pituitary insufficiency.
with in this
case would be exceptional for focal fatty change. Hepatic masses that may have sonographic, CT, and MR findings consistent
with
fat include
lipomas,
angiomyolipomas,
liver
cell adenomas, myelolipomas, and hepatocellular carcinomas with fatty metamorphosis [5]. In addition, some small (
