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Retroperitoneal Ganglioneuroma
①IlBong Kim, ②KiMan Lee, ③HongJin Kim, ④DongSug Kim***
①Dr. Kim ilbong Internal Medicine Clinic, Taegu, Korea,②DaeKyung Radiology Clinic, Taegu, Korea
epartment of Surgery, College of Medicine, Yeungnam Univ.,④Department of Pathology, College of Medicine, Yeungnam U
A 8-years-old girl visited clinic because of intermittent gross
hematuria for 3 days. At physical examination revealed no
specific abnormal findings. She had no personal history of
renal diseaes, pulmonary tuberculosis and drug abuse.
Laboratoty data : (1) CBC : Hb 12.8 gm%, WBC 4,400
/cmm, platelet 276 K/uL (2) U/A : colorless, PH 6.0, SG 1.025,
glucose (-), protein (-), blood (-), ketone (-), bilirubin (-), nitrite
Fig1.,Fig2.,Fig3. Ultrasonogram
(-), RBC 0-1 / HPF, WBC 0-1 / HPF (3) BUN 11.7 mg/dL,
glucose 121 mg/dL, total cholesterol 156 mg/dL, total protein 7
g/dL, albumin 4.5 g/dL, total bilirubin 0.5 mg/dL, ALP 1,001
U/L, LDH 403 U/L, ALT 20 U/L, AST 28 U/L, serum Na 145
mEq/L, K 4.3 mEq/L, Cl 108 mEq/L (4) prothrombin time 11.3
sec 97.8 %, aPTT 30.7 sec (5) ferritin and alpha-FP : normal,
bone marrow aspiration : normal (6) bone scan (MIBG) :
Fig4.,Fig5.,Fig6. Ultrasonogram
increased uptake (7) Urine VMA 2 mg/day.
Abdomen ultrasonogram reveals huge homogenous echoic
round mass (4.4 x 5.2 x 6.4 cm) in subhepatic region which is
growing or attached to liver. There is noted ovoid mass in right
adrenal gland. There are also noted multiple lymph nodes
enlargement in retrocaval and pericaval region. GB and biliary
tracts appear unremarkable. Both kidneys, spleen and
Fig7, Abdomen CT Fig8, Abdomen CT Fig9, Abdomen CT
pancreas appear unremarkable.
Impression : Huge mass in subhepatic region with right
adrenal gland metastasis and
multiple metastatic lymph
adenopathy in retrocaval and pericaval region.
->
Hepatoblastoma is suggested.
On Abdomen CT reveals huge relatively homogenous round
mass within liver. After contrast media injection, there was a
marked inhomogenous enhancement of mass within liver with
Fig10, Fig11, Fig12 Abdomen CT,
interior growing which size is 4.7 x 6.4 cm in dimension. There
shows displacement of portal vein and IVC due to
compression by mass. There shows multiple masses in
pericaval region and right adrenal gland mass. Pancreas and
both kidneys appear unremarkable. Pelvic organs appear
unremarkable.
Impression ; Hepatoblastoma with metastasis to right
adrenal gland and multiple
metastatic lymph nodes in
pericaval region.
Fig13, Abdomen CT
Fig14, Abdomen CT
The patient underwent laparatomy under general anesthesia.
An ill defined huge retroperitoneal mass was noted and
located at portal triad area. IVC, portal rein and CBD were
adhesed with mass. An incisional biopsy of main mass was
done and perigastric lymph nodes was excised.
Gross findings ; The submitted specimen consists of three
groups. The first specimen consists of pinkish gray polypoid
tissue, measuring 1.8 x 1.4 cm in dimension. The cut surface
shows whitish pink solid appearance. The second specimen
labeled as 8p lymph node consists of a piece of tissue. The
Fig15, Abdomen CT
Fig16, Gross Findings
third specimen labeled as 13th lymph node.
◆육안소견 : The cut surface of the retroperitoneal mass is solid
Microscopic findings : The retroperitoneal mass shows
and
scattered or grouped
abnormal ganglion cells and
yellow tan.
proliferation of schwann cells.
◆현미경소견 : The retroperitoneal mass shows scattered or
grouped abnormal ganglion cells and proliferation of schwann
Diagnosis ; I. Retroperitoneum, supra adrenal, right, cells.
incisional biopsy ;
Ganglioneuroma