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Weekly Chest CasesArchive of Old Cases

Case No : 1270 Date 2022-02-22

  • Courtesy of Mihyeon Park, Jin Young Yoo, Sung Jin Kim / Chungbuk National University Hospital
  • Age/Sex 41 / F
  • Chief ComplaintCough and sputum (onset: 2 weeks ago)
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Multifocal micronodular pneumocyte hyperplasia (MMPH) in tuberous sclerosis
Radiologic Findings
Fig 1-2. Chest CT scans show multifocal small nodules and GGNs in both lungs.
Fig 3-4. Chest CT scans show multifocal osteosclerotic change in T-spines.
Fig 5. Enhanced chest CT scan demonstrates a subendocardial focal fatty deposit in the interventricular septum.
Brief Review
Tuberous sclerosis complex (TSC) is an inheritable multiorgan disease. MMPH is a manifestation of TSC with a hamartomatous process of the lung that exhibits multiple tiny pulmonary nodules. Pathologically, it is characterized by the proliferation of type II pneumocystis along the alveolar septa. MMPH can occur in patients with or without lymphangioleiomyomatosis (LAM), predominantly in female patients.
The clinical manifestations resulting from isolated MMPH are dyspnea, cough, and mild to moderate hypoxemia. The clinical course of MMPH is usually not progressive and treatment is unnecessary.
Radiologically, MMPH presents as multiple nodules on CT. Nodules are 1-8mm in diameter and are diffusely scattered throughout the lung in a random distribution with regard to the secondary lobule. The differential diagnosis should also include a miliary granulomatous infection, Langerhans cell histiocytosis, hematogenous metastasis and multiple atypical adenomatous hyperplasia/adenocarcinoma in situ.
Additionally, the presence of well-circumscribed foci of homogeneous fat attenuation in the myocardium of patients with TSC was seen in the majority of patients with TSC usually located in the interventricular septum and the left ventricular wall. Fatty foci in patients with TSC appear to have unique CT characteristics with respect to location, attenuation, focality, absence of enhancement, and absence of invasive behavior that most closely resembles that of lipomas seen in other parts of the body.
In this patient, brain axial fluid-attenuation inversion-recovery image demonstrates focal areas of high signal intensity and enhanced CT image shows well-defined mass-like lesion with fat attenuation in the liver, compatible with angiomyolipoma. Considering all these features in the patient, these could be thought as manifestation of TSC.
References
1. Ristagno RL, Biddinger PW, Pina EM, Meyer CA. Multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis. AJR Am J Roentgenol 2005; 184:S37-39.
2. Adriaensen ME, Schaefer-Prokop CM, Duyndam DA, Zonnenberg BA, Prokop M. Fatty foci in the myocardium in patients with tuberous sclerosis complex: common finding at CT. Radiology 2009; 253:359-363.
3. Von Ranke FM, Faria IM, Zanetti G, Hochhegger B, Souza Jr AS, Marchiori E. Imaging of tuberous sclerosis complex: a pictorial review. Radiol Bras. 2017 Jan/Fev;50(1):48–54
Please refer to
Case 1124 Case 1050
Keywords

No. of Applicants : 96

▶ Correct Answer : 70/96,  72.9%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan HIROAKI ARAKAWA
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa General Hospital , Japan MASAFUMI KAIUME
  • - Ajou University Hospital , Korea (South) SUNG HYUN AN
  • - , United Kingdom SAMSON KADE
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - Osaka Red Cross Hospital , Japan YUSUKE UTSUNOMIYA
  • - IRSA LA ROCHELLE , France JEAN LUC BIGOT
  • - National Center of Neurology and Psychiatry , Japan MOTO NAKAYA
  • - , Italy PAOLO BALDASSARI
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - , Japan CHIAKI SATO
  • - Ichinomiya Nishi Hospital , Japan CHIHIRO KIGUCHI
  • - Ajou University Hospital , Korea (South) KYUNGMIN LEE
  • - Other , Korea (South) SEONGSU KANG
  • - Scans world , India PHILSON JOSEPH MUKKADA
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - The first affiliated Hospital of Xiamen University , China Qing Qiang Guo
  • - Korea University Ansan Hospital , Korea (South) HYO YOUNG KIM
  • - Osaka University , Japan AKINORI HATA
  • - Kaneda hospital , Japan HIROFUMI MIFUNE
  • - Ajou University Hospital , Korea (South) HAEIN LEE
  • - Shimada General Medical Center , Japan HAYATO NOZAWA
  • - , Korea (South) HYEWON CHOI
  • - Other , Korea (South) YEJI SHIN
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Ajou University Hospital , Korea (South) MINJI KIM
  • - Inje University Pusan Paik Hospital , Korea (South) JIYEON HAN
  • - Gifu University Hospital , Japan Yo Kaneko
  • - McGill , Canada MUTAZ ADNAN KHAIRO
  • - Chonnam National University Hwasun Hospital , Korea (South) MYUNG WON SONG
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - , Korea (South) YOU NA KIM
  • - , Korea (South) YEONG UK HWANG
  • - , United States HIROAKI TAKAHASHI
  • - Osaka City General Hospital , Japan SHU MATSUSHITA
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - , Japan MIDORI UENO
  • - Other , Korea (South) MINSU KIM
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Eskisehir Sehir Hastanesi , Turkey MURAT SAHIN
  • - , Qatar NABIL SHERIF MAHMOOD
  • - Osaka city university Hospital , Japan TATSUSHI OURA
  • - Azienda Ospedaliera di Cremona , Italy Pietro Sergio
  • - Other , Korea (South) SEONG WON JANG
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Manipal hospital Hebbal ,Bengaluru , India PRAVIN KUMAR M
  • - Asan Medical Center , Korea (South) BYUNGSOO IM
  • - Chonnam National University Hospital , Korea (South) KIM SANG GYUN
  • - Other , Korea (South) KYU-CHONG LEE
  • - Kyoto University Hospital , Japan SATOSHI IKEDA
  • - Paras hospital, Panchkula , India SHALEEN RANA
  • - Nishida Hospital , Japan SHOJI OKUDA
  • - Seoul National University Bundang Hospital , Korea (South) YOONAH DO
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Chonbuk National University Hospital , Korea (South) DONG HAN SHIN
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - TAKANA HAYASHI
  • - , Japan KAZUMA TERAUCHI
  • - Private sector , Greece VASILIOS TZILAS
  • - , Japan YUMI MAEHARA
  • - , Japan YUKI HAYASHI
  • - , Korea (South) WONJU HONG
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Hanyang University Hospital , Korea (South) SEUNG JIN YOO
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 2/96,  2.1%
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
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