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Adenoid Cystic Carcinoma Nasal Sudarmanto, Yohanes; Soehartono; Angelina, Aina; Yudhanto, Hendy; Putri, Anggie
Jurnal Klinik dan Riset Kesehatan Vol 1 No 2 (2022): Edisi Februari
Publisher : RSUD Dr. Saiful Anwar Province of East Java

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (858.04 KB) | DOI: 10.11594/jk-risk.01.2.8

Abstract

Latar belakang: Keganasan sinonasal memiliki angka kejadian yang jarang. Adenoid cystic carcinoma (ACC) merupakan tumor ganas tersering kedua di daerah sinonasal, memiliki karakteristik pertumbuhan lambat namun berpotensi metastasis jauh dan rekurensi yang tinggi. Tatalaksana utama penyakit adalah tindakan pembedahan radikal dilanjutkan radioterapi. Pemilihan pendekatan tindakan pembedahan bergantung pada stadium dan lokasi tumor. Prognosis keganasan sinonasal kurang baik, berhubungan dengan tipe histologis tumor, kejadian invasi perineural, perivaskular, dan metastasis jauh, lokasi tumor, usia pasien dan modalitas terapi. Tujuan: Melaporkan satu kasus ACC nasal dengan metastasis regional cervical yang dilakukan tindakan pembedahan dan dilanjutkan radioterapi. Laporan Kasus: Laki-laki, 70 tahun, datang ke klinik THTKL RSUD dr. Saiful Anwar Malang dengan keluhan benjolan di dalam hidung kanan disertai buntu hidung dan riwayat mimisan selama 1 tahun. Pasien dilakukan tindakan pembedahan dengan pendekatan rinotomi lateral menggunakan insisi Moure. Histopatologi jaringan operasi adalah ACC tipe cribiform. Kesimpulan: Tindakan pembedahan dilanjutkan radioterapi masih menjadi baku emas tatalaksanan ACC nasal. Evaluasi berkala jangka panjang harus dilakukan untuk deteksi dini rekurensi lokoregional dan kejadian metastasih jauh pasca tindakan pembedahan dan radioterapi.
Rare Case : Lymphangioleiomyomatosis in a 28 year-old woman with spontaneous bilateral pneumothorax Matabei, Shannon Conversia Pdabo; Djajalaksana, Susanthy; Chozin, Iin; Erawati, Dini; Wardhana, Koernia; Yudhanto, Hendy; Rahman, Perdana; Chen, Chung-Yu
Malang Respiratory Journal Vol. 7 No. 2 (2025): Volume 7 No 2, September 2025 Edition
Publisher : Universitaas Brawijaya

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.21776/ub.mrj.2025.007.02.04

Abstract

Introduction: Lymphangioleiomyomatosis (LAM) is an extremely rare disease with no known exact cause, in which there are abnormalities in the development of smooth muscle cells. This condition manifests in multiple organs, one of which is the lungs. Prevalence of LAM is 1 in 1,000,000, most commonly found in women of reproductive age. Pneumothorax occurs in two-thirds of LAM patients. It is possibly underdiagnosed in Indonesia. Case report: A 28-year-old woman came to the Emergency Department with sudden severe shortness of breath. Chest X-ray shows bilateral pneumothorax predominantly on the left side, and a chest tube was installed. Thorax MSCT revealed multiple cysts in all areas of lungs, while abdominal USG and MRI discovered masses in uterus and right adrenal. Left bullectomy-thoracotomy and lung biopsy were performed, with histopathology result supporting LAM. The patient was stable post surgery. LAM is associated with mutations in TSC1 or TSC2 genes, resulting in cystic changes caused by LAM cells proliferation in the lungs. In this patient, bilateral pneumothorax resulted from multiple subpleural cysts which eventually ruptured. Diagnosis of LAM was established by radiological imaging and lung biopsy. Surgical and non-surgical interventions were performed to address the acute manifestations of LAM. Unfortunately, Sirolimus which is the recommended long-term medication for LAM is not yet available in Indonesia. Conclusion: LAM is a rare disease. It’s important for clinicians to be familiar with this condition, aware of its progression and possible recurrence of pneumothorax, in order to provide appropriate interventions.