Interhospital Conference

History ผู้ป่วยเด็กชายไทยอายุ 11 ปี ภูมิลำเนา จ.กรุงเทพฯ Chief Complaint : รับมารักษาต่อด้วยเรื่องก้อนบริเวณต่อมทอลซิลด้านซ้าย 4 เดือน PTA

Present Illness 4 เดือน PTA ผู้ป่วยไปรักษาที่โรงพยาบาลเอกชนด้วยเรื่อง ไข้ เจ็บคอ ได้รับการวินิจฉัยว่าต่อมทอลซิลด้านซ้ายโต และอักเสบ แพทย์ให้การรักษาโดยการนอนโรงพยาบาล ให้ IV antibiotics, Left tonsillectomy 1 เดือนPTAแพทย์นัดติดตามอาการตรวจพบว่าก้อนโตขึ้นไม่มีอาการผิดปกติอย่างอื่น ผล pathology – no malignancy จึงส่งมารับการรักษาต่อที่โรงพยาบาลจุฬา

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 ก่อน 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 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 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 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 รับ consult at OPD ผู้ป่วยมีปัญหาเรื่องปวดที่ขาข้างขวาตั้งแต่ต้นขาถึงปลายขา ไม่มีปวดบริเวณอื่น 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 ผู้ป่วยยังมีอาการปวดขาไม่ดีขึ้น Bonefos (800) 2 cap oral OD เช้า

Progression 20/08/52 OPD Follow up, ปวดขามากโดยเฉพาะที่บริเวณน่อง, ซีดลง  admit for further investigation 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.

25/8/52 Start systemic treatment: Vinblastion, Prednisolone Progression 25/8/52 Start systemic treatment: Vinblastion, Prednisolone หลัง start systemic treatment ได้ 2 wk, อาการปวดลดลง และปวดห่างมากขึ้น Continue systemic treatment และสามารถcontrol painได้ด้วยยากิน D/C 15/9/52 then F/U as OPD case

Dipak at el, Clinical Medicine & Research, Volume 3, Number 2: 65-74 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 ภูมิลำเนา จ. แพร่ ผู้ป่วยมีอาการขาซ้ายบวมเป็น ๆ หาย ๆ ตั้งแต่อายุ 1 ปี 6 เดือน โดยบวมบริเวณสะโพกมาที่หัวเข่า ไม่แดงไม่ร้อน X-ray, U/S doppler, CT ปกติดี อายุ 9 ปีผู้ป่วยเริ่มมีปื้นแดงขึ้นที่บริเวณต้นขาซ้ายและสะโพกซ้ายจึงมารักษาที่โรงพยาบาลจุฬาลงกรณ์ 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 coagulogramปกติ

พฤษภาคม 2552 ผู้ป่วยเริ่มมีปื้นสีแดงขึ้นมาใหม่และมีเลือดออกไม่หยุดบริเวณ lesion ร่วมกับมีเลือดออกตามไรฟัน จึงมาโรงพยาบาล ระหว่าง admission ผู้ป่วยมีปัญหา 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

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A 40-year-old man admitted for right throat pain 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.

Primary tumors in the head and neck are very rare 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