Interhospital Conference

History

ผปวยเดกชายไทยอาย 11 ป ภมลำาเนา จ.กรงเทพฯ

Chief Complaint : รบมารกษาตอดวยเรองกอนบรเวณตอมทอลซลดานซาย 4 เดอน PTA

Present Illness

4 เดอน PTA ผปวยไปรกษาทโรงพยาบาลเอกชนดวยเรอง ไข เจบคอ ไดรบการวนจฉยวาตอมทอลซลดานซายโต และอกเสบ แพทยใหการรกษาโดยการนอนโรงพยาบาล ให IV antibiotics, Left tonsillectomy

1 เดอนPTAแพทยนดตดตามอาการตรวจพบวากอนโตขนไมมอาการผดปกตอยางอน ผล pathology – no malignancy จงสงมารบการรกษาตอทโรงพยาบาลจฬา

Past history : no underlying disease

no history of drug allergy

Family history : no malignancy

Physical ExaminationGA: A Thai boy, good consciousness, well

co-operativeHEENT: pink conjunctiva, anicteric sclera

Irregular mass below left tonsillar fossa, mass invaded posterior pillar and posterior pharyngeal wall, invade valleculae, but abutted epiglottis

LN : impalpableHeart: regular, normal S1S2, no murmurLungs: clear both lungsAbd: 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 ReportSection 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

ProgressionENT consult Tumor

conference Tumor conference suggest

MRI กอน plan management เพมเตม

PET/CT and MRI

PET/CT ReportPET/CT (03/07/52)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 ReportMultiple hypermetabolic bilateral

cervical lymph nodes (more on the left), likely nodal metastases.

Multiple pulmonary nodules, probably pulmonary metastases.

Multiple small hypermetabolic poorly osteolytic and non-osteolytic lesion, probably inhomogeneous marrow activity in child or foci of marrow infiltration. Correlation with other imaging modality such as bone scan is recommended

Bone ScanBone scan (17/08/52)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)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 spindle-shaped 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 รบ consult at OPD ผปวยมปญหาเรองปวดทขาขางขวาตงแตตนขาถงปลายขา ไมมปวดบรเวณอน 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 ผปวยยงมอาการ

ปวดขาไมดขน Bonefos (800) 2 cap oral OD เชา

Progression

20/08/52 OPD Follow up, ปวดขามากโดยเฉพาะทบรเวณนอง , ซดลง admit for further investigation Investigation : plain X-ray leg [right],

นด U/S right leg Consult pain: MO IV prn for pain Blood component as needed Continue Bonefos Continue Radiation

Progression

Follow up LabCBC: 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 Ultrasonography of the right calf (26/08/52)(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

หลง start systemic treatment ได 2 wk, อาการปวดลดลง และปวดหางมากขน

Continue systemic treatment และสามารถcontrol painไดดวยยากน

D/C 15/9/52 then F/U as OPD case

Gorham’s Disease

Dipak at el, Clinical Medicine &Research, Volume 3, Number 2:

-6574

A rare disorder characterizedby proliferation of vascular

channels that results in destru ction and resorption of osseou

s matrix. There have been fewer than150 cases reported in theliterature.

E tiology 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 s

imilar to a hemangioma or lymp hangioma. Wildly proliferating

neovascular tissue causes mass ive bone loss.

N o evidence of a malignant, neuropathic, or

infectious component involved in the causation o

f 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.I n some cases, pathologic fractur e often leads to its discovery.

Gorham’s disease can involve men or women and any age group

A lthough 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. T

he shoulder and the pelvis are the m ost common sites.

Clinical presentation

Treatment

The medical treatment for Gorham’s disease includes

-radiation therapy, anti osteocla stic medications (bisphosphona

-tes), and alpha2 b interferon. Surgical treatment options

include resection of the lesion and reconstruction using bone g

rafts and/or prostheses.

R adiation therapy are used for management of patients who have

large, symptomatic lesions with lo- ng standing, disabling functional i

nstability. Definitive radiation therapy in

moderate doses (40-45 Gy in2 Gy f ractions) appears to result in a goo

-d clinical outcome with few long te rm complications.

Treatment

In general, no single treatment modality has proven effective i

n arresting the disease.

The prognosis for patients with Gorham’s disease is generally

good unless vital structures ar e involved.

Treatment

CASE ผปวยชายอาย 20 ป ภมลำาเนา จ . แพร ผปวยมอาการขาซายบวมเปน ๆ หาย ๆ ตงแต

อาย 1 ป 6 เดอน โดยบวมบรเวณสะโพกมาทหวเขา ไมแดงไมรอน X-ray, U/S doppler, CT ปกตด

อาย 9 ปผปวยเรมมป นแดงขนทบรเวณตนขาซายและสะโพกซายจงมารกษาทโรงพยาบาลจฬาลงกรณ 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 syndromeTreatment กนยายน - 2541 พฤศจกายน 2542

- Prednisolone + Interferon alpha ตลาคม - 2542 มกราคม 2544- Vincristine clinical improve, platelet

count and coagulogramปกต

พฤษภาคม 2552 ผปวยเรมมปนสแดงขนมาใหมและมเลอดออกไมหยดบรเวณ lesion รวมกบมเลอดออกตามไรฟน จงมาโรงพยาบาล ระหวาง admission ผปวยมปญหา 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 105 9 93740. Sep; ( ): .

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 105 9 93740. Sep; ( ): .

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