Transcript Clinical Pathologic Conference

Clinicopathological Conference
Speaker: 楊凱堯 醫師
Supervisor: 林榮鈞 醫師
Division of Gastroenterology
Department of Internal Medicine
Tri-Service General Hospital
April, 1, 2016
07:30~08:30 A.M.
1
General data
Gender: Female
Age: 51
2
Chief complaint
Gradual abdominal fullness and edema of
lower limbs for 2 weeks.
3
Present illness
Ten months ago
Skin rash over four extremities and whole trunk
Received extensive treatment in many clinics (the dermatology,
family and rheumatology section), but poor response.
Six months ago
Hyperglycemia noted at other LMC
Two weeks ago
Gradual abdominal fullness
Edema of lower limbs in recent 2 weeks.
On presentation
Called at our GI OPD.
Body weight loss (57 --> 47 kgw in 10 months)
4
Past history
 Hx of past systemic disease:
Hyperglycemia for half a year under
diet control
 Hx of previous surgery: Denied
 Medications: steroids
 Allergy: NKA
5
Personal history
 Habit of cigarette smoking: Nil
 Habit of alcoholic drinking: Nil
 Habit of Betal-nuts chewing: Nil
 Social relationship: Well
 Occupation: housewife
 Denied recent traveling history
 Marital status: married
6
Family history
7
Physical examination
 BT: 36.1 ℃; PR: 98 bpm; RR: 18 cpm; BP: 133/80 mmHg
 Height: 157 cm; Weight: 51 kgw. BMI = 20.69.
 Skin: multiple well-demarcated erythematous plaques with fragile
vesicles and superficial scaling over four extremities, and whole trunk
 Head & neck: anemic conjunctivae.
 Abdomen: ovoid, shifting dullness(+).
 Extremities: pitting edema over lower legs, grade 2.
9
Lab data
檢驗項目
檢驗結果
單位
最小/最大安全值
WBC
5.81
103 /uL
4.50 – 11.00
RBC
3.64
106
Hgb
10.2
g/dL
12.0 – 16.0
HCT
30.8
%
38.0 – 47.0
MCV
84.6
fL
79.0 -100.0
MCH
28.0
Pg
25.4 - 34.6
MCHC
33.1
g/dL
Platelet
357
103 /uL
/uL
檢驗項目
檢驗結
果
單位
最小/最大安全
值
Glucose
137
mg/dL
74 – 109
BUN
13
mg/dL
6 – 20
Creatinine
0.5
mg/dL
0.5 – 0.9
AST
32
U/L
– 32
ALT
30
U/L
– 31
Sodium
135
mmol/L
136 – 145
30.0 - 37.0
Potassium
2.8
mmol/L
3.5 – 5.1
150 - 400
Chloride
102
mmol/L
98 – 107
Total calcium
8.9
mg/dL
8.6 – 10.2
Inorganic
Phosphate
4.2
mg/dL
2.7 – 4.5
3.90 - 5.50
12
Any Questions?
13
Problem lists
 Abdominal swelling.
 Edema of bilateral lower limbs.
 Unintentional body weight loss.
 Chronic, generalized figurate skin lesions.
 Hyperglycemia.
14
Problem lists
 Abdominal swelling.
 Edema of bilateral lower limbs.
 Unintentional body weight loss.
 Chronic, generalized figurate skin lesions.
 Hyperglycemia.
15
Causes of abdominal swelling
As the six Fs:
 Flatus
 Increased intestinal gas.
 Fat
 Imbalance between caloric intake and energy expenditure associated with a
poor diet and sedentary lifestyle; it also can be a manifestation of certain
diseases, such as Cushing’s syndrome.
 Fluid
 The accumulation of fluid within the abdominal cavity (ascites).
 Fetus
 Pregnancy results in increased abdominal girth. The uterus moves from the
pelvis into the abdomen.
 Feces
 Severe constipation or intestinal obstruction, often accompanied by
abdominal discomfort or pain, nausea, and vomiting.
 Fatal growth (often a neoplasm)
 Enlargement of the intra-abdominal organs (ex: hepatomegaly, splenomegaly
or an abdominal aortic aneurysm), urinary bladder distention, malignancies,
abscesses, or cysts can grow to sizes that lead to increased abdominal girth.16
Harrisons Principles of Internal Medicine, 19th Edition
Problem lists
 Abdominal swelling.
 Edema of bilateral lower limbs.
 Unintentional body weight loss.
 Chronic, generalized figurate skin lesions.
 Hyperglycemia.
17
Cause of leg edema
24 hour urine
Protein
182
mg
檢驗項目
檢驗結果
單位
最小/最大安全值
BUN
13
mg/dL
6 – 20
Creatinine
0.5
mg/dL
0.5 0.9
Total protein
5.4
g/dL
6.6 - 8.7
Albumin
2.6
g/dL
3.97 – 4.94
19
Leg edema: clinical clues to the differential diagnosis. Geriatrics.1993 May;48(5):34-40, 45
20
Harrisons Principles of Internal Medicine, 19th Edition
Cause of leg edema
 Hypoproteinemia / Hypoalbuminemia
 Drug reactions (Glucocorticoids)
 Hepatic cirrhosis
 Lymphedema
21
Problem lists
 Abdominal swelling.
 Edema of bilateral lower limbs.
 Unintentional body weight loss.
 Chronic, generalized figurate skin lesions.
 Hyperglycemia.
22
Cause of unintentional weight loss
23
Harrisons Principles of Internal Medicine, 19th Edition
Cause of unintentional weight loss
 Endocrine and metabolic
 Rheumatologic disease
 Gastrointestinal disorders
 Cancer
 Infections
24
Harrisons Principles of Internal Medicine, 19th Edition
Endocrine data
Endocrine and metabolic
- DM
- Adrenal insufficiency
檢驗代碼 檢驗結果 檢驗結果單位
最小/最大安全值
Cortisol
Morning 7-28
Afternoon 2-18
Stimulated >= 18
Supressed < 2
13.57
ug/dL
25
RIA data
26
Tumor marker
GYN Ultrasonography:
- Uterus: A/V, 5.4x4.2x3 cm. ET:3.4 cm
- ROV:1.8 cm
- LOV:2 cm
- Unremarkable malignancy noted in uterus, ovary
27
UGI PES
29
Abdomen sonography
1. Massive ascites
2. Chronic liver parenchyma disorder
3. Heterogenous hyperechoic mass lesion over bilateral lobes of liver
30
Abdomen CT
Radiologist
31
Problem lists
 Abdominal swelling.
 Edema of bilateral lower limbs.
 Unintentional body weight loss.
 Chronic, generalized figurate skin lesions.
 Hyperglycemia.
33
Figurate skin lesions
34
Harrisons Principles of Internal Medicine, 19th Edition
Problem lists
 Abdominal swelling.
 Edema of bilateral lower limbs.
 Unintentional body weight loss.
 Chronic, generalized figurate skin lesions.
 Hyperglycemia
35
Cause of hyperglycemia
36
Harrisons Principles of Internal Medicine, 19th Edition
Final differential diagnosis
 Pancreas tumor (primary?) & multiple liver tumors (metastases?)
 Ascites, cause to be determined, DDx: Hypoalbuminemia
 Edema of bilateral lower limbs, DDx: Hypoalbuminemia,
Glucocorticoids, Lymphedema
 DM, type??
 Chronic, generalized figurate skin lesions, DDx: Primary ??
Systemic ??
38
Diagnostic procedure
Diagnostic abdomen paracentesis with
ascites cytology
EUS-FNA for pancreatic tumor (unavailable)
Sono-guided liver biopsy
Skin biopsy
39
請教感染科醫師…
40
請教皮膚科醫師…
這個病人有慢性皮膚症狀表現，有
哪些鑑別診斷? 除皮膚切片檢查，
需要做其它檢查?
41
請教新陳代謝科醫師…
這個病人有胰臟腫瘤，抽血檢查
有高血糖情形，請問有哪些鑑別診
斷?需要做其它檢查?
42
Diagnostic procedure
Diagnostic abdomen paracentesis with
ascites cytology
Sono-guided liver biopsy
Skin biopsy
43
Pathologist
44
Ascites studies
45
SAAG: 2.6-1.8=0.8 < 1.1g/dL
Differential diagnosis of ascites
46
Harrisons Principles of Internal Medicine, 19th Edition
51
Sleisenger and Fordtran‘s Gastrointestinal and Liver Disease 10th edition
Chapter 33, 501-541.e15
52
Glucagon level
檢驗項目
檢驗結果 單位
Glucagon
26874
最小/最大安全值
pg/ml <100
55
Final diagnosis
Neuroendocrine carcinoma of pancreas
with multiple hepatic metastases and intraabdominal carcinomatosis with massive
ascites, grade II, with glucagon secretion,
cT2N0M1, stage IV, ECOG PS:1-2
56
Review
57
Incidence of NETs
Incidence
No 2 small intestine
No 3 rectum
No 8 Pancreas
Sleisenger and Fordtran‘s Gastrointestinal and Liver Disease 10th edition
Chapter 33, 501-541.e15
58
59
Sleisenger and Fordtran‘s Gastrointestinal and Liver Disease 10th edition
Chapter 33, 501-541.e15
60
Sleisenger and Fordtran‘s Gastrointestinal and Liver Disease 10th edition
Chapter 33, 501-541.e15
61
Sleisenger and Fordtran‘s Gastrointestinal and Liver Disease 10th edition
Chapter 33, 501-541.e15
Summary of glucagonoma
 Nearly all reported cases of the glucagonoma syndrome have been
associated with tumors originating in the alpha cells of the pancreas.
 Glucagonomas are generally slow-growing, but usually advanced by
the time of diagnosis.
 The clinical syndrome classically associated with glucagonoma
includes necrolytic migratory erythema (NME), cheilitis, diabetes
mellitus, anemia, weight loss, diarrhea, venous thrombosis, and
neuropsychiatric symptoms. Weight loss and NME are the most
prevalent symptoms, occurring in approximately 65 to 70 percent of
patients by the time of diagnosis.
 While not pathognomonic for glucagonoma, the presence of NME
should prompt further work-up for a pancreatic neuroendocrine
tumor.
Stephen E Goldfinger MD: Glucagonoma and the glucagonoma syndrome,
UpToDate Aug 11, 2015
62
Summary of glucagonoma
 Glucagonoma is associated with markedly elevated serum
concentrations of glucagon (>500 pg/mL).
 Many conditions other than glucagonoma can induce "physiologic"
elevations in the serum glucagon concentration. These include
hypoglycemia, fasting, trauma, sepsis, acute pancreatitis, abdominal
surgery, Cushing's syndrome, and renal and hepatic failure.
However, these conditions are associated with only moderate
elevations of glucagon, usually less than 500 pg/mL (upper limit of
normal <100 pg/mL).
 In patients suspected of having a glucagonoma, cross sectional
imaging with helical, triple-phase abdominal computed tomography
(CT) is the initial imaging procedure of choice. Since the primary
tumor is usually large by the time of diagnosis, it is localizable by CT
in the majority of cases.
 As a result of its greater sensitivity for liver metastases, some
physicians prefer MRI over CT for assessing the status of the liver.
Stephen E Goldfinger MD: Glucagonoma and the glucagonoma syndrome,
UpToDate Aug 11, 2015
63
Summary of glucagonoma
 Once the primary tumor and/or metastases have been localized by CT or
EUS, we proceed with needle biopsy to confirm the diagnosis. EUSguided biopsy is the preferred method for localized disease.
 In comparison, a CT-guided liver biopsy is performed if liver metastases
are present. For the minority of cases in which the tumor is localized at the
time of diagnosis, resection of the primary pancreatic tumor is indicated
since it offers the chance of complete cure.
 Treatment of patients with metastatic disease may include resection of the
primary tumor and metastases, treatment with a somatostatin analog,
embolization, radiofrequency or cryoablation, and chemotherapy.
 The prevalence of metastatic disease at the time of diagnosis varies from
series to series, ranging from 50 to 100 percent. The most common site of
metastasis is the liver, followed by regional lymph nodes, bone, adrenal
gland, kidney, and lung.
 Once the tumor is metastatic, cure is rarely, if ever, achieved. However,
despite the presence of metastases, many patients are able to experience
prolonged survival with a combination of therapeutic modalities.
Stephen E Goldfinger MD: Glucagonoma and the glucagonoma syndrome,
UpToDate Aug 11, 2015
64
Post Tx skin lesion f/u
One month Later
65
Thanks for your attention
66