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Abstrak :
Latar Belakang: Prevalensi kanker di Indonesia meningkat menjadi 1,8 per 1000 penduduk pada 2018. Diagnosis dini yang tepat dibutuhkan untuk mengurangi angka mortalitas. Salah satu cara diagnosis tumor berupa pemeriksaan penanda tumor, seperti vanillylmandelic acid (VMA). Penanda tumor tersebut termasuk metabolit katekolamin yang akan meningkat produksinya pada beberapa tumor neuroendokrin. Kadar katekolamin sendiri dapat dipengaruhi oleh usia dan jenis kelamin. Belum ada data proporsi VMA positif dalam urin pada pasien dugaan tumor neuroendokrin di Jakarta serta hubungan VMA dalam urin dengan usia dan jenis kelamin. Tujuan: Tujuan dari penelitian ini adalah untuk mengetahui proporsi VMA positif dalam urin pasien dugaan tumor neuroendokrin di Jakarta serta hubungannya dengan usia dan jenis kelamin. Metode: Penelitian ini menggunakan desain penelitian cross-sectional. Data sekunder dikumpulkan berupa lembar hasil serta formulir pemeriksaan VMA pasien dugaan tumor neuroendokrin pada periode 2010 hingga April 2019. Data didapatkan dari Departemen Biokimia dan Biologi Molekuler, FKUI. Pengambilan data sekunder dilakukan pada Oktober 2019 dengan total subjek penelitian 295. Pemeriksaan kualitatif VMA dalam urin dilakukan dengan metode spot test. Hasil pemeriksaan positif menunjukkan kadar VMA dalam urin > 8 mg/24 jam, sedangkan hasil negatif menunjukkan kadar VMA dalam urin  8 mg/24 jam. Kriteria inklusi berupa data subjek dengan diagnosis sementara neuroblastoma, pheochromocytoma, dan paraganglioma. Hasil: Proporsi VMA positif dalam urin pasien dugaan tumor neuroendokrin dalam penelitian ini adalah 14,2% (IK95%, 10,2 – 18,2%). Analisis hubungan VMA dalam urin dengan usia memberikan hasil p 0,023. Analisis hubungan VMA dalam urin dengan jenis kelamin menunjukkan hasil p 0,885. Kesimpulan: Terdapat hubungan antara VMA dalam urin dengan usia dan tidak terdapat hubungan antara VMA dalam urin dengan jenis kelamin.

Kata kunci: Vanillylmandelic Acid, Tumor Neuroendokrin, Neuroblastoma, Pheochromocytoma, Paraganglioma. ......Background: Cancer prevalence in Indonesia increased to 1.8 per 1000 population in 2018. Early diagnosis is needed to reduce mortality rate. One of the ways to diagnose tumors is by examining tumor markers, such as vanillylmandelic acid (VMA). VMA is catecholamine metabolites which will increase their production in several neuroendocrine tumors. Catecholamine level can be influenced by age and gender. There is no data about proportion of positive VMA in urine of patients with suspected neuroendocrine tumors in Jakarta and the association of VMA in urine with age and gender. Objective: The objective of this study was to determine the proportion of positive VMA in urine of patients with suspected neuroendocrine tumors and its association with age and gender. Methods: This study used a cross-sectional study design. Secondary data were collected in the form of VMA examination forms and result sheets from patients with suspected neuroendocrine tumors in the period 2010 to April 2019. Data were obtained from the Department of Biochemimstry and Molecular Biology, FKUI. Collection of secondary data conducted in October 2019 with a total of 295 study subjects. Qualitative examination of urinary VMA used spot test method. Positive examination result  showed levels of VMA in urine >8mg/24 hours, while negative result showed levels of VMA in urine  8mg/24 hours. Inclusion criteria were subject data with a provisional diagnosis of neuroblastoma, pheochromocytoma, and paraganglioma. Results: The proportion of positive VMA in urine of suspected neuroendocrine tumor patients in this study was 14,2% (CI95%, 10,2 – 18,2%). Analysis of the association between VMA in urine and age result was p value 0,023. P value form analysis of the association between VMA in urine and gender was 0,885. Conclusion: There is an association between VMA in urine with age and there is no association between VMA in urine with gender.

Keywords: Vanillylmandelic Acid, Neuroendocrine Tumors, Neuroblastoma, Pheochromocytoma, Paraganglioma.

Abstrak :
Co-occurrence of cyanotic congenital heart disease (CCHD) and phaeochromocytoma (PCC) and paraganglioma (PGL) are rare, although some cases have been reported. We report a case of left paraganglioma in a 20-year-old lady with an underlying CCHD who underwent palliative Glenn shunt, subsequently developed polycythaemia and cavernous sinus thrombosis presented with palpitation, sweating, headache and hypertension of 3-months duration at the age of 17. The abdominal CT scan revealed an enhancing left paraaortic mass measuring 5.2 cm x 4.4 cm x 3.8 cm. A 24-hour urine catecholamine demonstrated raised noradrenaline level to six times upper limit of normal and hence diagnosis of left sympathetic (sPGL) was made. In view of the delayed diagnosis and significant morbidity associated with her condition, surgical treatment is no longer an option. Therefore, vigilant screening and early treatment of PCC-PGL in patients with CCHD are crucial in order to avoid significant morbidity and ensure a good quality of life.