Interhospital Conference

1. Interhospital Conference

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

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

4. Past history : no underlying disease no history of drug allergy • Family history : no malignancy

5. 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

6. 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

7. PROBLEM LISTS

8. INVESTIGATION

9. Tonsillar Biopsy

10. 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

11. Pathological Report

12. 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

13. Progression ENT consult Tumor conference Tumor conference suggest MRI ก่อน plan management เพิ่มเติม

14. PET/CT and MRI

19. 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.

20. 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

21. 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.

24. X-Ray

29. 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

30. Pathological report of Bone Biopsy

32. 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

33. Pathology Report Right proximal femur : no definite vascular tumor

34. Impression : Gorham disease

35. 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

36. 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

37. 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

38. 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)

40. 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.

41. 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

42. Gorham’s Disease Dipak at el, Clinical Medicine & Research, Volume 3, Number 2: 65-74

43. 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.

44. 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.

45. No evidence of a malignant, neuropathic, or infectious component involved in the causation of this disorder.

46. 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.

47. 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. Clinical presentation

48. 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.

49. 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

50. 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. Treatment