Weekly Chest CasesArchive of Old Cases

Case No : 1154 Date 2019-12-15

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  • Courtesy of Dong Jin Im / Severance Hospital, Yonsei University College of Medicine
  • Age/Sex 27 / F
  • Chief ComplaintIntermittent atypical chest pain
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Multifocal micronodular pneumocyte hyperplasia (MMPH)
Radiologic Findings
Figure 1. Chest PA shows nodular opacity in left upper lung field.
Figure 2-3. CT scans show well-defined subsolid nodule in left upper lobe apicoposterior segment (25 mm) and other multiple smaller GGOs in bilateral lungs (5-13 mm).
Brief Review
MMPH is a rare pulmonary disorder that can be associated with Tuberous Sclerosis (TS). It is characterized by multicentric, well-demarcated nodular proliferation of type II pneumocytes along alveolar septa. Since the hamartomatous nature of micronodular epithelial proliferations has been emphasized, the terms multiple adenomatoid tumors, acinar atypical adenomatoid proliferation of epithelium, and micronodular hyperplasia of type II pneumocytes have been proposed as being more descriptive names. Recently, these lesions have come to be commonly called MMPH, taking their pathogenesis and locations into account. MMPH can occur in patients with or without lymphangioleiomyomatosis (LAM), predominantly in female patients. Clinically, patients with MMPH may present with dyspnea, cough, and mild to moderate hypoxemia. Unlike with pulmonary LAM, treatment is usually unnecessary because MMPH does not appear to be fatal and progressive.
At thin-section CT, multiple tiny nodules (3-10 mm) or numerous miliary nodules (1-3 mm) are diffusely scattered throughout the lung in a random distribution. Although differentiation of MMPH from miliary metastatic or granulomatous disease is difficult, MMPH should be considered in the differential diagnosis when multiple tiny pulmonary nodules are present in patients with TS.
This patient did not know about TS. After pathologic confirmation of lung lesion, TS was confirmed in gene test.
Please refer to
Case 979, Case 1124,
KSTR imaging conference 2018 Spring  Case 15,
References
1. Ross L. Ristagno, Paul W. Biddinger, Elsira M. Pina and Cris A. Meye, Multifocal Micronodular Pneumocyte Hyperplasia in Tuberous Sclerosis. American Journal of Roentgenology. 2005;184: S37-S39
2. Umeoka S, Koyama T, Miki Y, Akai M, Tsutsui K, Togashi K., Pictorial review of tuberous sclerosis in various organs. Radiographics. 2008 Nov-Dec;28(7):e32
Keywords
lung, multiple micronodules, MMPH,

No. of Applicants : 40

▶ Correct Answer : 10/40,  25.0%
  • - CH de la Cote Basque , France PAUL ARDILOUZE
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Ajou University Hospital , Korea (South) HYERIN KIM
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - National Center for Global Health and Medicine , Japan HIROSHI TAKUMIDA
  • - , Japan KAZUMA TERAUCHI
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - Chonnam National University Hwasun Hospital , Korea (South) WONGI JEONG
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 12/40,  30.0%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Chungbuk National University Hospital , Korea (South) YEONGTAE PARK
  • - , Korea (South) CHOHEE KIM
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
  • - University of Tsukuba Hospital , Japan SODAI HOSHIAI
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