pseudorheumatoid nodule in the liver of an adolescent male
TRANSCRIPT
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Pseudorheumatoid nodule in the liver of anadolescent male
Shehzad A. Saeeda,*, David R. Kellyb, William D. Hardin Jr.c
aDepartment of Pediatrics, Division of Pediatric Gastroenterology and Nutrition Sciences,
Children’s Hospital and the University of Alabama at Birmingham, Birmingham, AL 35233, USAbDepartment of Pathology and Laboratory Medicine, Children’s Hospital and the University of Alabama at Birmingham,
Birmingham, AL 35233, USAcDepartment of Pediatric Surgery, Children’s Hospital and the University of Alabama at Birmingham, Birmingham,
AL 35233, USA
0022-3468/$ – see front matter D 2006
doi:10.1016/j.jpedsurg.2006.04.036
* Corresponding author. Tel.: +1 205
E-mail address: [email protected]
Index words:Pseudorheumatoid nodule;
Focal nodular hyperplasia;
Meckel diverticulum;
Deep granuloma annulare
Abstract Focal hepatic lesions in the pediatric population are rare and are usually found on incidental
imaging of the abdomen. The most common lesions are focal nodular hyperplasia and hemangioma, but
the differential diagnosis is quite broad. We present a case of an adolescent male who was found to have
a large pseudorheumatoid nodule of the liver that was successfully resected. Only one previous case is
reported in the literature and none in the pediatric age group.
D 2006 Elsevier Inc. All rights reserved.
1. Case
A 14-year-old male presented with vague complaints of
right-sided chest pain. Persistence of these complaints
necessitated an evaluation that included a chest computed
tomography (CT) scan, which revealed a space-occupying
lesion greater than 3 cm in the right lobe of the liver. A
contrast CT scan of the abdomen demonstrated a 4.0� 4.0�3.3 cm enhancing lesion in the posterior segment of the right
lobe of the liver. Delayed imaging showed filling of the
central region of the lesion, suggestive of a hemangioma. A
tagged red blood cell (RBC) scan was, however, negative. A
technetium sulfur colloid scan, done to evaluate for the
presence of focal nodular hyperplasia, was also normal. The
patient’s physical examination was normal, with no evi-
dence of hepatosplenomegaly, subcutaneous nodules, or
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u (S.A. Saeed).
rashes. Laboratory evaluation revealed a normal complete
blood count with no evidence of hemolysis and a normal
prothrombin time and a-fetoprotein concentration. Liver
function studies (alanine aminotransferase [ALT]; aspartate
aminotransferase [AST], alkaline phosphatase, albumin, and
bilirubin concentrations) were normal.
An exploratory laparotomy was subsequently performed,
and a nodular, well-demarcated lesion was removed from the
right lobe of the liver (Fig. 1). A Meckel diverticulum, found
incidentally during routine examination of the bowel, was
resected. Cholecystectomy was also performed. Pathologic
evaluation of the liver specimen showed a circumscribed
3.8 � 3.1 � 3.0 cm nodular mass that was surrounded by
normal appearing liver parenchyma. Histologically, the
mass consisted of small granulomas surrounded by a rim
of scarred, remodeled nodular liver parenchyma. Most of
the granulomas were stellate-shaped and had a necrobiotic
appearance characterized by palisading histiocytes with a
sprinkling of lymphocytes surrounding central areas of
Journal of Pediatric Surgery (2006) 41, 1479–1482
Fig. 1 Circumscribed nodular mass in the right lobe of the liver.
S.A. Saeed et al.1480
fibrinoid necrosis (Fig. 2). Necrotic collagen and reticulin
fibers coursed through the center of the granulomas
(Fig. 3). A Masson trichrome stain demonstrated coarse
collagen bands separating small rounded liver cell nodules
adjacent to the more centrally located granulomas (Fig. 4).
Many of the collagen bands contained clusters of proli-
ferated bile ductules. The liver parenchyma adjacent to the
mass was normal, with preservation of the acinar
architecture, liver cell cords, and sinusoids. There was
no significant alteration of the portal tracts or blood ves-
sels. Special stains for fungi, the cat scratch organism
(Bartonella henselae), syphilis, mycobacteria, and para-
sites were negative.
An evaluation for rheumatologic disease was negative.
The patient had normal antinuclear antibody and rheumatoid
factor titers but an elevated erythrocyte sedimentation rate
(52 mm/h), which was normal (7 mm/h) when repeated
Fig. 2 A, One of many necrobiotic granulomas within the liver mass. B
in one of the granulomas (hematoxylin-eosin, �33 [A] and �132 [B]).
2 months later and remained normal through the subsequent
serial measurements. A liver function panel checked
2 months after resection was normal with normal ALT,
AST, and albumin concentrations.
The patient did well over the course of the next 2 years
before presenting with postprandial epigastric and upper
abdominal pain associated with episodic loose stools and
occasional blood per rectum with hard stools. Biochemical
evaluation (complete blood count, amylase, lipase, erythro-
cyte sedimentation rate, C-reactive protein, antinuclear
antibody, gamma glutamyltranspeptidase, liver function
studies, Prometheus IBD serologies [perinuclear antineutro-
philic cytoplasmic antibody, anti-Saccharomyces cerevisiae
antibody, and a-anti–outer membrane of porin C], and stool
studies) was normal. A minimally elevated indirect bilirubin
concentration (2 mg/dL) was noted and correlated with a
paternal history of jaundice during periods of stress. An
upper gastrointestinal small bowel follow through and a
magnetic resonance imaging of the abdomen were also
normal. Upper and lower endoscopic evaluation revealed
reflux esophagitis and normal colonic biopsies with no
evidence of chronic inflammatory bowel disease. The patient
has done well on Proton Pump Inhibitor therapy (Prevacid,
TAP Pharmaceuticals, Inc, Lake Forest, Ill) and antispas-
modic treatment, with normal weight gain and resolution of
abdominal pain symptoms.
2. Discussion
We believe that this is the first reported pediatric case of
hepatic pseudorheumatoid nodule. One case of a hepatic
rheumatoid nodule has been previously reported [1]. That
case was an autopsy report of a 54-year-old woman with
, Palisading histiocytes surround a central area of fibrinoid necrosis
Fig. 3 Necrotic collagen (coarse blue fibers) (A) and reticulin (coarse black fibers) (B) extend through the center of one of the granulomas
(A: Masson trichrome, �132; B: Gordon and Sweet reticulin, �132).
Case report: pseudorheumatoid nodule in the liver of an adolescent male 1481
rheumatoid arthritis of several years’ duration who died as
result of complications from chronic renal failure, amyloid-
osis, and septicemia. Liver histology revealed a hemangi-
oma and multiple discrete nodules with a central zone of
necrosis, histiocytic proliferation at the periphery, fibrosis,
and chronic inflammation. The histologic features were
similar to several concurrent subcutaneous nodules. Our
case differs in that there are no associated subcutaneous
lesions, and no evidence of rheumatologic disease has been
found so far.
Our patient presented with nonspecific symptoms and
was found to have an incidental hepatic mass. Evaluation
of the liver histology was unremarkable for the usual
Fig. 4 Scarring with nodule formation (A, B) and bile duct prolif
(hematoxylin-eosin, �33 [A, B] and �132 [C]).
entities associated with granulomatous inflammation. The
histologic features were also not consistent with focal
nodular hyperplasia (normal blood vessels and portal tracts
as well as absence of lymphocytic infiltration). The patient
had no fevers, chest radiographs did not show hilar
adenopathy, and cultures and special stains were negative
for mycobacteria, cat scratch disease, syphilis, parasites,
and fungi. Special stains (S-100 protein, CD1a) were also
negative for Langerhans cell histiocytosis. Serologies were
normal for autoimmune/rheumatological and inflammatory
bowel diseases. Radiological evaluations (abdominal CT,
magnetic resonance imaging, and barium studies) have
shown no evidence of primary hepatobiliary or gastroin-
eration (C) in the liver immediately adjacent to the granulomas
Table 1 Differential diagnosis of hepatic granulomatoid
lesions
Infections Medications
Viral Fungal Allopurinol
Cytomegalovirus Histoplasmosis Carbamazepine
Epstein-Barr virus Cryptococcosis Halothane
Varicella Candidiasis Hydralazine
Viral hepatitis Coccidioidomycosis Phenylbutazone
Phenytoin
Bacterial Parasitic Quinidine
Mycobacteria
Mycobacterium
avium-intercellulare
complex
Brucellosis
Tularemia
Whipple disease
Granuloma inguinale
Psittacosis
Yerseniosis
Leprosy
Leprosy
Schistosomiasis
Toxoplasmosis
Visceral larva
migrans
Visceral
leishmaniasis
Rickettsial
Q fever
Spirochetal
Syphilis
Sulfonamides
Miscellaneous
Sarcoidosis
Crohn disease
Chronic
granulomatous
disease
Wegener
granulomatosis
Hepatic diseases
Primary biliary
cirrhosis
Reactive hepatitis
Granulomatous
hepatitis
Fatty liver
S.A. Saeed et al.1482
testinal disease. An incidental note was made of minimal
indirect hyperbilirubinemia in the absence of signs of
hemolysis on the peripheral blood smear, features sugges-
tive of Gilbert syndrome.
Typical lesions of pseudorheumatoid nodule (also known
as deep or subcutaneous granuloma annulare [DGA]) are
soft tissue nodules found on the tibial, scalp, forearm, or
periorbital regions in children [2,3]. These children seldom
have rheumatoid disease. The pathology is characterized by
subcutaneous location, necrobiosis, mild eosinophilic infil-
tration, and palisading mononuclear cellular infiltrate. In
addition to DGA, other entities to consider in the differential
diagnosis for a palisading granulomatous lesion include
rheumatoid nodule of rheumatic fever, juxtaarticular rheu-
matoid nodule of rheumatoid arthritis, and necrobiosis
lipoidica diabeticorum. None of these entities are common
in children, and none have been identified in our patient.
The granulomatous lesions in the liver are not consistent
with the typical features of hepatic granulomas [4,5], and
the clinical features and evaluation for most of these
etiologies are negative (Table 1).
To our knowledge, this is the first case report of a
pseudorheumatoid nodule in the liver of a pediatric patient
who currently has no clinical features of a rheumatologic
disease. We plan to follow the patient closely to determine
the natural history of this entity.
References
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[2] McDermott MB, Lind AC, Marley EF, et al. Deep granuloma annulare
(pseudorheumatoid nodule) in children: clinicopathologic study of 35
cases. Pediatr Dev Pathol 1998;1:300-8.
[3] Evans MJ, Blessing K, Gray ES. Pseudorheumatoid nodule (deep
granuloma annulare) of childhood: clinicopathologic features of twenty
patients. Pediatr Dermatol 1994;11:6 -9.
[4] Denk H, Scheuer PJ, Baptista A, et al. Guidelines for diagnosis
and interpretation of hepatic granulomas. Histopathology 1994;25:
209 -18.
[5] Irani SK, Dobbins III WO. Hepatic granulomas: review of 73 patients
from one hospital and survey of the literature. J Clin Gastroenterol
1979;1:131-43.