Transcript Case Conference I

Interhospital
Conference
History
ผู้ป่วยเด็กชายไทยอายุ 11 ปี ภูมิลาเนา จ.กรุ งเทพฯ
Chief Complaint : รับมารักษาต่ อด้ วยเรื่องก้ อนบริเวณ
ต่ อมทอลซิลด้ านซ้ าย 4 เดือน PTA
Present Illness
4 เดือน PTA ผู้ป่วยไปรั กษาที่โรงพยาบาลเอกชนด้ วย
เรื่อง ไข้ เจ็บคอ ได้ รับการวินิจฉัยว่ าต่ อมทอลซิลด้ านซ้ าย
โต และอักเสบ แพทย์ ให้ การรักษาโดยการนอน
โรงพยาบาล ให้ IV antibiotics, Left tonsillectomy
1 เดือนPTAแพทย์ นัดติดตามอาการตรวจพบว่ าก้ อนโต
ขึน้ ไม่ มีอาการผิดปกติอย่ างอื่น ผล pathology – no
malignancy จึงส่ งมารับการรักษาต่ อที่โรงพยาบาลจุฬา
Past history : no underlying disease
no history of drug allergy
Family history : no malignancy
Physical Examination
GA: A Thai boy, good consciousness, well co-operative
HEENT: pink conjunctiva, anicteric sclera
 Irregular mass below left tonsillar fossa, mass invaded posterior pillar
and posterior pharyngeal wall, invade valleculae, but abutted
epiglottis
LN : impalpable
Heart: regular, normal S1S2, no murmur
Lungs: clear both lungs
Abd: normoactive, no hepatosplenomegaly
Investigation
CBC Hb 11.6g/dl, Hct 35.7%, WBC 5,680 cells.mm3
(N59.9%, L32.9%, M4.2%, E 2.5%, B0.5%), Plt 200,000
cells/mm3
Blood chemistry
 BUN 12 mg/dl, Cr 0.53mg/dl
 Electrolyte: Na 138 mmol/L, K 4.7mmol/L, Cl 99 mmol/L, CO2 28
mmol/L
 TB 0.23 mg/dl, DB 0.02 mg/dl, SGOT 19mg/dl, SGPT 22mg/dl, ALP
194 U/L
PROBLEM LISTS
INVESTIGATION
Tonsillar Biopsy
Management
Intraoral biopsy (28/04/52)
 Finding: Irregular mass at left tonsillar fossa,
invaded posterior pillar, posterior pharyngeal
wall, valleculae, abutted epiglottis
 Operation: partial excision about 50% by
electrocautery
Pathological Report
Pathology Report
Section of mucosa show vascular lesion infiltrating in
underlying stroma. There are lined by plump endothelial
cells witch have round to spindle nuclei, vascular
chromatin, distinct nuclei and moderate amount of
eosinophilic cytoplasm. Mitoses are frequently seen.
Intervening stroma reveals hyalinization. Hemorrhage
and many chronic and acute inflammatory cells infiltrate
are observed. Covering mucosa display focal ulcer with
fibrinous exudate and acute inflammatory cells infiltrate.
Pseudoepitheliomatous hyperplasia is occationally noted.
Hemangioendothelioma
Progression
ENT consult Tumor conference
Tumor conference suggest MRI ก่ อน plan management
เพิ่มเติม
PET/CT and MRI
PET/CT Report
PET/CT (03/07/52)
A 2.1x2.2 cm hypermatabolic irregular rim enhancing
mass with central hypodensity at left palatine tonsillar
fossa, consistent with history of hamangioendothelioma.
This is possible residual disease
Mild focal bulging medical contour of right palatine tonsil
without definite abnormal enhancing area, showing
homogeneous FDG accumulation, small focal lesion
cannot be excluded. Tissue diagnosis is recommended.
PET/CT Report
Multiple hypermetabolic bilateral cervical lymph nodes
(more on the left), likely nodal metastases.
Multiple pulmonary nodules, probably pulmonary
metastases.
Multiple small hypermetabolic poorly osteolytic and nonosteolytic lesion, probably inhomogeneous marrow
activity in child or foci of marrow infiltration. Correlation
with other imaging modality such as bone scan is
recommended
Bone Scan
Bone scan (17/08/52)
Bone lesion at skull is corresponding with multiple
geographic lytic lesions without sclerotic rim and some
blastic lesions in diploic space of bilateral parietal bone,
likely bone metastases.
Bone lesion at C2 vertebral body, pelvic rim, right
acetabulum, head, proximal, mid shaft and distal right
femur, likely due to bone metastases as correlated with
lytic lesion seen on PET/CT.
X-Ray
Management
Due to PET/CT found multiple small
hypermetabolic poorly osteolytic and non-osteolytic
lesion, suspected bone metastases  consult
orthopaedics 23/7/52 Excision Bone at Right iliac,
right proximal tumor
Pathological report of
Bone Biopsy
Pathology Report
Right iliac bone biopsy (23/07/52)
Section show fragments of bony tissue and marrow
element. Few pieces of bone reveal proliferation of blood
vessels with occasionally lined by round to spindleshaped cells. Cells have round nuclei, fine chromatin,
visible nucleoli
Immunohistochemistry: epithelioid
hemangioendothelioma
Pathology Report
Right proximal femur : no definite vascular tumor
Impression : Gorham disease
Progression
03/08/52 Start Radiotherapy at tonsils and lymph node
70 Gy
06/08/52 รับ consult at OPD ผู้ป่วยมีปัญหาเรื่องปวดที่ขา
ข้ างขวาตัง้ แต่ ต้นขาถึงปลายขา ไม่ มีปวดบริเวณอื่น
 CBC: Hb 8.8 g/dl , WBC11,800 cells/mm 3 (N 78.3
%, L14.9 %, M3.6% , E2.9% , B0.3% ), Platelet
122,000 cells/mm 3
 LD-PRC 300 ml IV drip in 3 hour
Progression
7/8/52 CBC: Hb 11 g/dl , WBC 9,850 /mm 3 (N 66 %,
L21 %, M 3% , E 7% , AL 3% ), Platelet 104,000 /mm 3
PTT 36.8 sec [30.4] PT 15.4 sec [13.4]
Fibrinogen 641 mg/dl
D-dimer 0.2 mcg/dl [< 0.3]
10/08/52 OPD Follow up ผู้ป่วยยังมีอาการปวดขาไม่ ดขี นึ ้
Bonefos (800) 2 cap oral OD เช้ า
Progression
20/08/52 OPD Follow up, ปวดขามากโดยเฉพาะที่
บริเวณน่ อง, ซีดลง  admit for further investigation
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Investigation : plain X-ray leg [right], นัด U/S right leg
Consult pain: MO IV prn for pain
Blood component as needed
Continue Bonefos
Continue Radiation
Progression
Follow up Lab
 CBC: Hb 10.1g/dl , WBC 4,360 cells/mm3
(N76%, L12%, M5%, E6%, AL1%), Plt
62,000cells/mm3
 Fibrinogen = 4.66 G/L (1.7-4.0)
 D-dimer Vidas =3,322 ng/ml (<500)
Ultrasound
Ultrasonography of the right calf (26/08/52)
Normal attenuation of muscles and subcutaneous tissue
of right calf. No fluid or space taking lesion within right
calf is observed. The color Doppler ultrasound show on
evidence of hypervascularity or abnormal vascular
formation within right calf.
Progression
25/8/52 Start systemic treatment: Vinblastion,
Prednisolone
หลัง start systemic treatment ได้ 2 wk, อาการปวดลดลง
และปวดห่ างมากขึน้
Continue systemic treatment และสามารถcontrol painได้
ด้ วยยากิน
D/C 15/9/52 then F/U as OPD case
Gorham’s Disease
Dipak at el, Clinical Medicine & Research, Volume 3,
Number 2: 65-74
A rare disorder characterized by proliferation of
vascular channels that results in destruction and
resorption of osseous matrix.
There have been fewer than 150 cases reported
in the literature.
Etiology of Gorham’s disease remains poorly
understood
The pathological process is the replacement of
normal bone by an aggressively expanding but
non-neoplastic vascular tissue similar to a
hemangioma or lymphangioma. Wildly proliferating
neovascular tissue causes massive bone loss.
No evidence of a malignant, neuropathic, or
infectious component involved in the causation of
this disorder.
Clinical presentation
The clinical presentation of Gorham’s disease is
variable and depends on the site of involvement.
Gorham’s disease may complain of dull aching
pain or insidious onset of progressive weakness.In
some cases, pathologic fracture often leads to its
discovery.
Clinical presentation
Gorham’s disease can involve men or women and any
age group
Although most cases are discovered before the age of
40 years.
No familial predisposition has been found.
The process may affect the appendicular or the axial
skeleton. The shoulder and the pelvis are the most
common sites.
Treatment
The medical treatment for Gorham’s disease
includes radiation therapy, anti-osteoclastic
medications (bisphosphonates), and alpha-2b
interferon.
Surgical treatment options include resection of the
lesion and reconstruction using bone grafts and/or
prostheses.
Treatment
Radiation therapy are used for management of
patients who have large, symptomatic lesions with
long-standing, disabling functional instability.
Definitive radiation therapy in moderate doses
(40-45 Gy in 2 Gy fractions) appears to result in a
good clinical outcome with few long-term
complications.
Treatment
In general, no single treatment modality has
proven effective in arresting the disease.
The prognosis for patients with Gorham’s disease
is generally good unless vital structures are
involved.
CASE
ผู้ป่วยชายอายุ 20 ปี ภูมิลาเนา จ. แพร่
ผู้ป่วยมีอาการขาซ้ ายบวมเป็ น ๆ หาย ๆ ตัง้ แต่ อายุ 1 ปี
6 เดือน โดยบวมบริเวณสะโพกมาที่หวั เข่ า ไม่ แดงไม่ ร้อน
X-ray, U/S doppler, CT ปกติดี
อายุ 9 ปี ผู้ป่วยเริ่มมีปื้นแดงขึน้ ที่บริเวณต้ นขาซ้ ายและ
สะโพกซ้ ายจึงมารักษาที่โรงพยาบาลจุฬาลงกรณ์ PE:
multiple discrete purplish nodules on skin covering,
swelling at both inguinal area, left buttock, left thigh,
back
CBC : Hb 12.6 g%, WBC 6,000 /mm3 [N 56%, L 28 %, atypical L
13%, Mo 3 %] platelet 349,000 /mm3
PT 13.1 sec [11.3], PTT 38.9 sec [33.3]
Impression :Kasabach Meritt syndrome
Treatment กันยายน 2541- พฤศจิกายน 2542
- Prednisolone + Interferon alpha
ตุลาคม 2542- มกราคม 2544
- Vincristine  clinical improve, platelet count and coagulogram
ปกติ
 พฤษภาคม 2552 ผู้ป่วยเริ่มมีปื้นสีแดงขึน้ มาใหม่ และมี
เลือดออกไม่ หยุดบริเวณ lesion ร่ วมกับมีเลือดออกตาม
ไรฟั น จึงมาโรงพยาบาล ระหว่ าง admission ผู้ป่วยมี
ปั ญหา hemothorax both lungs, compression fracture at
T12 and multiple osteolytic lesion at spine and rib
Treatment vincristine weekly [total 7 doses] vinblastine
INF alfa2b [ 45 doses]
Radiation 10 fraction
THANK YOU
FOR YOUR ATTENTION
A case report of epithelioid hemangioendothelioma
metastasizing to the tonsil
A 40-year-old man admitted for right throat pain
he underwent radical surgery.
Epithelioid hemangioendothelioma was first diagnosed
Lung specimens at open biopsy 4 years earlier showed
the same histological features indicating he had had
epithelioid hemangioendothelioma lesion since that time.
We assumed this epithelioid hemangioendothelioma had
originated in the lung and metastasized to the right tonsil
Nippon Jibiinkoka Gakkai Kaiho. 2002 Sep;105(9):937-40.
Epithelioid hemangioendothelioma is an extremely rare,
difficult-to-diagnose vascular tumor mainly originating
from the lung or liver.
Primary tumors in the head and neck are very rare
Nippon Jibiinkoka Gakkai Kaiho. 2002 Sep;105(9):937-40.
Differential Diagnosis for Tonsillar
hypertrophy
 Infectious Disorders (Specific Agent)
 Infectious mononucleosis
 Infected organ, Abscesses
 Pharyngitis
 Adenoiditis, acute
 Tonsillitis/exudative, acute
 Tonsillitis, chronic
 Neoplastic Disorders
 Tonsil lymphosarcoma
 Tonsil, Lymphoepithelioma
 Metabolic, Storage Disorders
 Tangier's disease
 Hereditary, Familial, Genetic Disorders
 Lipodystrophy, generalized
 Reference to Organ System
 Adenoid hypertrophy
 Tonsillar hypertrophy syndrome