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Weekly Chest CasesArchive of Old Cases

Case No : 1079 Date 2018-07-02

  • Courtesy of Dabee Lee, MD., Kye Ho Lee, MD. / Dankook University Hospital
  • Age/Sex 49 / F
  • Chief ComplaintChest pain (intermittent, onset: a few months ago), No previous medical history
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Mesenchymal cystic hamartoma
Radiologic Findings
Figs 1-1 ~ 1-3. Chest CT scan shows multiple well-defined nodules and thin-walled cysts in both lungs.
Fig 2. On mediastinal window setting, nodules show internal low attenuation.
Fig 3-1 ~ 3-2. After 6 months, some nodules were unchanged, but in other nodules there was a change in size or cystic portion..
Brief Review
Mesenchymal cystic hamartoma of the lung (MCH) is a very rare disease with an indolent clinical course that is predominantly comprised of immature mesenchymal cells. Imbalance in growth of the endodermal respiratory epithelium and the mesodermal vascular elements, leads to the formation of nodules and cysts.
Although MCH may be detected in infancy, 3 or 4 decades usually elapse before it comes to clinical attention. The reported age range is from 1.5 to 53 years and the male-female ratio is 4:11.
Imaging shows propagation of new nodules and enlargement of cysts multiple cysts or nodules in most patients. The cysts were 1 to 10 cm in diameter, whereas the nodules were several millimeters to 1 cm. The nodules and cysts represent different stages of the same disease with the nodules occurring first. As the nodules become bigger, especially after reaching a diameter greater than 1 cm, the nodules usually transform into cysts. The cysts and nodules are more commonly seen in a bilateral distribution, but the lesions may be unilateral at the earlier stages.
MCH shows benign course in most cases. In some cases, hemorrhage from the systemic arteries into the cysts causes hemoptysis. Other serious complications include pneumothorax or hemothorax from rupture of a subpleural cyst and very rarely malignant transformation (mesenchymal sarcoma).
MHC needs to be differentiated from the other conditions which have similar clinicoradiographic presentations like pleuropulmonary blastoma, cystic adenomatoid malformation and lymphangiomyomatosis.
A surgical procedure is probably necessary to correct complications and establish the diagnosis; however routine surgical resection is not recommended due to the multicentric and usual benign nature of the disease. Close surveillance is recommended due to the potential of malignant transformation.
References
1. Fasanya AA, Hattab Y, Patel A, Lega M. Respir Med Case Rep. 2017 May 12;21:158-160.
2. Young Uk Lee, Jang Hoon Lee, Jong Hyun Baek. Korean J Thorac Cardiovasc Surg. 2016 Aug; 49(4): 302–305.
3. Zhu H, Huang S, Zhou X. Ann Thorac Surg. 2012 Jun;93(6):e145-7.
Keywords
Lung, Neoplasm_benign,

No. of Applicants : 78

▶ Correct Answer : 1/78,  1.3%
  • - Srp , Romania SILVIU MIHAIL DUMITRU
▶ Correct Answer as Differential Diagnosis : 4/78,  5.1%
  • - Chungbuk National University Hospital , Korea (South) YEONGTAE PARK
  • - Samsung Medical Center , Korea (South) MIN YEONG KIM
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
  • - Chonnam National University Hospital , Korea (South) YU BIN LEE
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Current Editor : Sung Shine Shim, MD, PhD. Email : sinisim@ewha.ac.kr

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