CONTOH Laporan kasus 18 tahun wanita dengan tumor intraabdomen

Laporan Kasus

1. 18 YEARS OLD FEMALE WITH INTRAABDOMINAL DESMOID TYPE FIBROMATOSIS TUMOR DIVISION OF HEMATOLOGY AND MEDICAL ONCOLOGY DEPARTMENT INTERNAL MEDICINE MEDICAL FACULTY HASANUDDIN UNIVERSITY 2021 Jumiati Satrul Sahyuddin Saleh CASE REPORT

2. VISI Menjadi pusat pendidikan yang unggul , mandiri dan bermartabat untuk menghasilkan Dokter Spesialis Ilmu Penyakit Dalam yang berkualitas dan mampu bersaing secara regional, nasional maupun global pada tahun 2025, dengan didukung oleh sumber daya manusia yang professional dan bertanggung jawab .

3. MISI Menyelenggarakan Pendidikan di bidang Ilmu Penyakit Dalam dan riset Memberikan pelayanan Kesehatan di bidang Ilmu Penyakit Dalam dengan pendekatan kultural dan budaya secara paripurna dan bermutu Meningkatkan kuantitas dan kualitas penelitian dasar dan aplikatif Ilmu Penyakit Dalam yang bertaraf Internasional Menciptakan sistem manajemen Program Studi Ilmu Penyakit Dalam yang transparan , akuntabel , responsible, independent, terintegrasi dan berkeadilan

4. 1. INTRODUCTION

5. DESMOID TUMOR (AGGRESSIVE DEEP-SEATED FIBROMATOSIS) These tumors are often considered a chronic disease and require careful observation, because these tumors have a risk of mortality due to aggressive local invasion. DESMOID TUMOR A rare disease of fibrous tissue proliferation , the term desmoid was first described by Mueller in 1838, based on the morphology of the myofibroblastic cells which form the desmoid tumors . Ma JH, Ma ZH, Dong XF, Yin H, Zhao YF. Abdominal wall desmoid tumors: A case report. Oncol Lett. 2013;5(6):1976-1978. doi:10.3892/ol.2013.1297 Howard JH, Pollock RE. Intra-Abdominal and Abdominal Wall Desmoid Fibromatosis. Oncol Ther . 2016;4(1):57-72. doi:10.1007/s40487-016-0017-z On General Population , DE S MOID TUMOR IS RARE, Accounts for 0.03% of All Neoplasms and Less 3% of Soft Tissue Tumors . A number of study shows 37-50% Desmoid tumors start in the ABDOMEN , and are more common in WOMEN than men with a ratio of 2:1

6. Name : Ms. M Age : 18 years old Address : Polmas Occupation : - Religion : Moeslem Ethnic : Buginese Marriage Status : Single Hospital : RSWS Reg. N umber : 921558 CASE REPORT

7. Chief complaint: abdominal pain Current Medical History: Abdominal pain since 1 month ago, c omplaint of an abdomen lump for 7 m onths . The lump first appeared with an initial size of a chicken egg, then the size progress to the size of a tennis ball . No history of weight loss . No fever and history of fever before. Nausea or vomiting (-). History of menarche at aged 15. CASE REPORT

8. PHYSICAL EXAMINATION VITAL SIGN

9. CASE REPORT T horax: I : Symmetrical, left is the same as the right P : No pain. The tumor mass is not palpable. P : Percussion is sonor at hemithorax bilateral A : broncho vesicular breathing, no ronch i , no wheezing. Heart: I : Ictus cordis is not appears . P : Ictus cordis is not palpable . P : dullness sound, cardiac border within normal limit A : Regular S1-S2 heart sounds , no gallops, no murmurs PHYSICAL EXAMINATION

10. CASE REPORT Abdomen: I : convex , follow breathing movements. Scar tissue at midline to lateral sinistra of umbilicus A: N ormal Intestinal peristal tic . P: Liver are not palpable . spleen are not palpable , tumor mass is palpable , solid, tenderness (+) P: Tympani Extremities : no edema PHYSICAL EXAMINATION

11. CASE REPORT 11/02/21 WBC 5.200 NEUT 57,1 LYMP 23,8 MONO 11,4 EO 14,6 BASO 0,6 HGB 12,9 HCT 37 MCV 85 MCH 29 MCHC 35 PLT 351.000 Pemeriksaan Hasil Nilai Rujukan GDS 102 140 mg/dl Ureum 10 10-50 mg/dl Kreatinin 0,56 L : <1,3 P:<1,1 mg/dl S GOT 19 <3 8 U/L SGPT 13 41 U/L Natrium 142 136-145 mmol/L Kalium 4,2 3,5-5,1 mmol/L Albumin 3,6 3,5 – 5 HBsAg Non reactive Non reactive Anti HCV Non reactive Non reactive

12. TIMELINE SEPT 11 TH , 2020 D/ Adnexa Mass NON-CONTRAST MSCT OF WHOLE ABDOMEN IN RSWS Interpretation: Left Psoas Abscess Suggestive of TB SEPT 24 th , 2020 Biopsy: Benign Cyst D/ INTRAABDOMINAL TUMOR R/ LAPAROTOMY EXPLORATORY OCT 19 th , 2020 D/ INTRAABDOMINAL TUMOR R/ LAPAROTOMY + TUMOR DEBULKING PA: DESMOID TUMOR FIBROMATOSIS TYPE NOV 2020- JAN 2021 D/ DESMOID TUMOR R/ RADIOTHERAPY 25 CYCLE FEB 2021 HOM CONSULTATION D/ DESMOID TUMOR FIBROMATOSIS TYPE PLAN: IHC VIMENTIN AND CYTOKERATIN, IHC ER, ECHOCARDIOGRAPHY APR-SEPT 2021 FULL COURSE CHEMOTHERAPY PLAN: CONTROL CT SCAN

13. CASE REPORT NON-CONTRAST MSCT OF WHOLE ABDOMEN RSWS Interpretation: Left psoas abscess suggestive of TB that extends to the retroperitoneal and pelvic cavities, pushing the left kidney and left ureter cranially as well as abdominal aorta and left iliac artery to the right. Sept 14 th , 2020

14. CASE REPORT HISTOPATHOLOGY EXAMINATION OF TISSUE FROM ABDOMINAL TUMOR (24/09/2020) Interpretation: Desmoid Type Fibromatosis Yellow box (Long fascicles that infiltrate fat tissue)

15. CASE REPORT HISTOPATHOLOGY EXAMINATION OF TISSUE FROM ABDOMINAL TUMOR Interpretation: Desmoid Type Fibromatosis Obj 10x (Yellow arrows: perivascular edema)

16. CASE REPORT HISTOPATHOLOGY EXAMINATION OF TISSUE FROM ABDOMINAL TUMOR Interpretation: Desmoid Type Fibromatosis Obj 10x (Yellow arrow: Blood vessel Blue arrow: collagen stroma )

17. CASE REPORT HISTOPATHOLOGY EXAMINATION OF TISSUE FROM ABDOMINAL TUMOR Interpretation: Desmoid Type Fibromatosis Obj 40x Lymphocyte cell infiltration

18. CASE REPORT IHC FEB 24 TH , 2021 Interpretation: Positive Vimentin, Negative Cytokeratin. Immunohistochemical indicate a soft tissue tumor

19. Before chemotherapy After chemotherapy

20. 3. DISCUSSION A CASE REPORT: A FEMALE AGED 18 DIAGNOSED WITH INTRA-ABDOMINAL DESMOID TYPE FIBROMATOSIS TUMOR

21. Desmoid Tumor is also known as Aggressive Fibromatosis . These tumors grow as a result of abnormal proliferation of monoclonal myofibroblasts . The microscopic appearance usually shows benign characteristic but with biological characteristic of in between fibrous lesions and sarcomas. The exact etiology of Desmoid Tumors is unknown , but it is believed to be multifactorial with genetic, endocrine and physical factors playing important roles in the development and growth of the tumor. Risk factors include High Estrogen Conditions (Pregnancy) and Antecedents Trauma, or Germline Predispositions such as FAP Syndrome. Case: 18 years old female The associations with female gender and reproductive age, a history of abdominal surgery or trauma and family history of fibromatoses are well described. Disease more frequently occurs between puberty and fourth decade of life. Pregnancy Case: The mass of the left psoas muscle for extending to the pelvic cavity IAD: mesentery or retroperitoneum AWD: muscle fascia or the abdominal/chest wall EAD: neck, shoulders, upper limbs, gluteal region Bui N, Kummar S. Molecular insights into desmoid tumors . Oncotarget . 2017;8(53):90608-90609. doi:10.18632/oncotarget.21293 DISCUSSION

22. Bui N, Kummar S. Molecular insights into desmoid tumors. Oncotarget . 2017;8(53):90608-90609. doi:10.18632/oncotarget.21293 WNT and Notch Signaling Pathway DISCUSSION

23. Tabel 2. Desmoid Tumor and Immunohistochemistry of Gene in patients with Desmoid tumor Leithner A, Gapp M, Radl R, et al. Immunohistochemical analysis of desmoid tumours . J Clin Pathol . 2005;58(11):1152-1156. doi:10.1136/jcp.2005.026278 DISCUSSION Case: ER negative In the Immunohistochemical study on Desmoid Tumors conducted by Leithner et al., the results show that all samples were negative for Estrogen a Receptors, HER2, and Progesterone Receptors.

24. Gounder MM, Mahoney MR, Van Tine BA, et al. Treatment ofDesmoid Tumors in 2019. N Engl J Med. 2018;379(25):2417-2428. doi:10.1056/nejmoa1805052 DISCUSSION Management of Desmoid Tumor Is complex and requires multidisciplinary approach with several specific skills. Active surveillance should be considered early in most cases. Patients need to be explained about the characteristics of the disease and should be observed to minimize the effect of therapy on the patient's quality of life. The management of the disease should not cause more discomfort than the disease itself Figure 7. Diagnosis and Management of Desmoid Tumor

25. NCCN Therapeutic Strategies for Desmoid Tumors. DISCUSSION

26. NCCN Therapeutic Strategies for Desmoid Tumors. DISCUSSION Doxorubicin-based chemotherapy has been effective in patients with recurrent or unresectale tumors Systemic therapy using NSAID, hormonal or biological agents, or cytotoxic drugs have shown promising results in patients with desmoid tumors.

27. CONCLUSION A case of symptomatic Intraabdominal Desmoid type fibromatosis tumors in Young Women with No History of Trauma, Pregnancy and AFP has been reported. The patient underwent surgical intervention. However, after surgical intervention and radiotherapy, the tumor relapse so patient underwent 6 cycles Doxorubicin and Dacarbazine regimens chemotherapy. There is good clinical outcome after chemotherapy

28. CASE REPORT PRESENTATION “THANK YOU”