<Chief
Complaint>
Progressive left flank dull pain for two weeks
<Brief
History> A 76-year-old man
visited emergent department because of progressive left flank
pain for two weeks. The pain was dull in character and was
persistent. It was not associated with postural change and
there were no radiation nor obvious aggravating and relieving
factors. He experienced malaise, poor appetite, and body
weight loss of 5Kg in one month. There were no hematuria and
dysuria. He had history of urolithiasis and underwent
extracorporeal shock wave lithotripsy (ESWL) two years ago. He
denied history of psychiatric illness, smoking, drinking, or
illicit drug use. The stool passage was normal. He was
admitted to urology ward under the suspicion of urolithiasis.
However, two episodes of hematemesis (about 150-200ml fresh
blood in each episode) happened on the 4th day of
hospitalization. Ketoprfen (intramuscular) was ever given
three times for pain control during hospitalization.
Gastroenterologists were consulted for further evaluation and
management.
<Physical
Examination> On consultation,
physical examination revealed a thin but well-developed man
with acute ill-looking. Heart rate was 114 bpm, temperature
was 36.7 ℃, blood pressure was 106/70 mmHg. Conjunctiva was
pale and a 1.5cm hard lymphadenopathy was noted at
submandibular region. Auscultation of the abdomen showed
hyperactive bowel sounds. There was no shifting dullness or
hepatosplenomegaly. Knocking pain was noted at left flank
area.
<Laboratory and Image
Study>
1. CBC/DC
WBC |
RBC |
MCV |
MCHC |
Hb |
Hct |
PLT |
K/μL |
M/μL |
fL |
g/dL |
g/dL |
% |
K/μL |
6.2 |
3.83 |
80.6 |
31.2 |
8.1 |
31.6 |
141 |
Seg |
Mono |
Eos |
Baso |
Lym |
% |
% |
% |
% |
% |
70.4 |
7 |
3 |
0 |
20
|
2. Biochemistry
BUN |
Cr |
Na |
K |
AST |
ALT |
Glu |
CRP |
mg/dL |
mg/dL |
meq/L |
meq/L |
U/l |
U/l |
mg/dL |
mg/dL |
30 |
2.0 |
138 |
4.1 |
26 |
30 |
106 |
1.4 |
3. Urine analysis:
Appearance |
Sp.gr |
PH |
Protein |
Glu |
Ketone |
Clear |
1.006 |
6.0 |
-- |
-- |
-- |
OB |
Urobilinogen (EU/dL) |
WBC (/HPF) |
RBC (/HPF) |
Cast (/LPF) |
Crystal (/LPF) |
-- |
0.1 |
50-60 |
3-5 |
-- |
-- |
Abdominal plain film: normal.
Renal
ultrasonography: enlargement of bilateral kidneys and
mild hydronephrosis of left kidney.
Ureteroscopic
examination: narrowing of bilateral ureteral lumens
without urolithiasis nor tumor.
Double J tube
was inserted to the left ureter.
<Course and
Treatment> Emergent upper
endoscopy revealed multiple doughnut–like tumors at gastric
body and antrum (Figure
1A , Figure1B).
Giant gastric folds were also noted. As the bleeding was
minimal (from gastric tumors) during endoscopy and there were
no visible bleeding vessels in the stomach and duodenum,
endoscopic hemostasis was not done. The bleeding stopped after
NPO and intravenous omeprazole injection. Computed tomography
(CT) of abdomen and pelvis showed perirenal mass enveloping
bilateral kidneys and segmental bowel wall thickening at
jejunum (Figure 2A
, Figure2B).
There was neither lymphadenopathy nor mass lesion compressing
the ureters. Small bowel barium study showed segmental filling
defects with thumb-printing appearance at jejunum and ileum
(Figure
3
).
However, acute renal
failure progressed (serum urea and creatinine levels elevated
to 59.4 mg/dL and 4.0 mg/dL, respectively). Histological
evaluation of the gastric biopsy specimen revealed
intermediate to large-size lymphoid cells crowding between
glands in a classical starry-sky pattern. The cellular
proliferation rate was extremely high, which was identified by
nearly 100% Ki-67 positivity. Special stains for tumor cells
showed CD20(+), CD21(-), and CD3(-). These findings were
compatible with Burkitt’s lymphoma. Tumor cells were negative
for Epstein-Barr virus (EBV) encoded RNAs. Serology test
showed positive for EBV-VCA IgG and negative for human
immunodeficiency virus (HIV). Both the urine cytology and
cerebral spinal fluid (CSF) analysis disclosed lymphoblastic
cells. Bone marrow aspiration and biopsy did not show marrow
involvement of lymphoma.
Systemic chemotherapy
with rituximab, BCNU, vincristine, methotrexate, etoposide,
and methylprednisolone and intrathecal injection of
methotrexate were administered after establishing the
diagnosis of Burkitt's lymphoma. After two courses of systemic
chemotherapy and intrathecal injection of methotrexate, his
general condition improved remarkably and nearly complete
remission was noted by follow-up panendoscopy, endoscopic
ultrasonography, small bowel series, CT scan of abdomen, and
cerebral spinal fluid cytoloty. Dramatic normalization of
renal function was also noted soon after initiation of
chemotherapy. Unfortunately, the patient died of pneumonia
during the 3rd course of chemotherapy.
<Analysis>
此病人的胃腫瘤的特色為多發性甜甜圈狀的腫瘤,同時可見持續性少量的滲血。這類型腫瘤的鑑別診斷包括Burkitt's
淋巴瘤,轉移至胃的腫瘤(常見的有黑色素瘤、肺癌、乳癌等),Gastrointestinal stromal
tumor(GIST),或Kaposi's肉瘤等等。胃鏡下胃的distensibility還不錯,同時看到有巨大胃皺摺,電腦斷層及小腸攝影發現小腸亦有區段性增厚。頸部亦有淋巴結結腫大,加上LDH升高,而其他腫瘤指數正常,故臨床診斷為淋巴瘤,而病理檢查更進一步診斷為Burkitt's淋巴瘤。胃出血常見的原因包括消化性潰瘍、食道或胃之靜脈瘤或Mallorg-Weiss症候群。胃腫瘤引起的大量出血較不常見,胃淋巴瘤有15-30﹪會發生上消化道出血,但是僅有少數病例引發大量吐血。有些報告指出胃腫瘤引起之出血可利用內視鏡治療達到止血的效果。但在這個病人其胃腫瘤之出血是廣泛的、緩慢性的滲血,亦無看到明顯的血管,所以並未利用內視鏡進行止血治療。在支持性療法及化學治療之後,病人未再發生上消化道出血。
Burkitt's 淋巴瘤是Non-Hodgkin's
lymphoma(NHL)的其中一種,占NHL的1-2﹪,最早是由Dennis Burkitt
在1958年時所發表,他發現烏干達的小孩易患有這類的淋巴瘤,它們腫瘤細胞生長得相當快速,而且,對化學治療的反應相當的好,但病人的預後較一般淋巴瘤差。後來在美洲也有發現Burkitt's
淋巴瘤的病例,但發生率較低,且好發在腸胃道及腹部淋巴結,與EB-virus的關聯性也較小。Burkitt's
淋巴瘤約有30-50﹪會侵犯到腎臟,常見的影像學包括單個或多個腎臟腫瘤,或瀰漫性腎臟浸潤,或從腎臟周圍包住腎臟(此病人的電腦斷層顯示為此類)。在治療方面,目前仍以化學治療為主,此病人使用之處方為R-BOMES(Rituximab
BLNU ,Vincristine ,Methotrexate,Etoposide and
methylprednisolone)治療後二個月,胃、小腸、頸部以及腎臟之病變幾乎完全消失。當發現多發現胃腫瘤時,應仔細找尋是否有淋巴結腫大或其他器官,如皮膚、肺部、乳房等等是否有腫瘤。腫瘤指數、胸部X光、腹部超音波掃描、電腦斷層掃描、上、下消化道及小腸攝影等檢查均可幫忙進一步診斷。
References:
- Collins J, et al.: Gastroenterology
1983; 85:425-429.
- Sharma et al.: Am J Hematol 2001;
67:48-50.
- Priebe WM: Gastrointest Endosc 1986;
32:352-354.
- Miyaguchi S, et al.: Endoscopy 1992;
24:603.
- Kadakia SC, et al.. Am J
Gastroenterol 1992;
87:1418-1423.
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