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  Table of Contents    
CASE REPORT  
Year : 2014  |  Volume : 57  |  Issue : 1  |  Page : 92-93
Multifocal pulmonary mucinous cystic neoplasm with ovarian type stroma, a new finding in an extremely rare case report


1 Department of Pathology; Transplant Research Center, Shiraz University of Medical Sciences Shiraz, Iran
2 Department of Surgery, Shiraz University of Medical Sciences Shiraz, Iran
3 Department of Pathology, Shiraz University of Medical Sciences Shiraz, Iran

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Date of Web Publication17-Apr-2014
 

   Abstract 

Primary pulmonary mucinous cystic neoplasm is a rare tumor and there are only a few case reports in the English literature. Herein we report a very unusual multifocal pulmonary mucinous neoplasm. Microscopic sections revealed ovarian type stroma which was diffusely positive for estrogen receptor, same as its pancreaticobiliary counterpart. To the best of our knowledge such a presentation of pulmonary mucinous neoplasm has not been previously reported in the English literature.

How to cite this article:
Geramizadeh B, Ziyaian B, Khaleghi S. Multifocal pulmonary mucinous cystic neoplasm with ovarian type stroma, a new finding in an extremely rare case report. Indian J Pathol Microbiol 2014;57:92-3

How to cite this URL:
Geramizadeh B, Ziyaian B, Khaleghi S. Multifocal pulmonary mucinous cystic neoplasm with ovarian type stroma, a new finding in an extremely rare case report. Indian J Pathol Microbiol [serial online] 2014 [cited 2019 Dec 7];57:92-3. Available from: http://www.ijpmonline.org/text.asp?2014/57/1/92/130910



   Introduction Top


Primary pulmonary mucinous cystic neoplasm is an extremely rare tumor and after its first report in 1969 by Eck et al. very rare case reports have been published under different names such as mucinous cyst, mucinous cystadenoma, mucinous cystic tumor and etc. [1]

Herein we report a rare case of multifocal primary pulmonary mucinous cystic neoplasm with ovarian type stroma in a 47-year-old female patient presented with the chief complaint of cough and dyspnea since 11 months prior to admission.

Her past medical history showed diabetes mellitus and hypertension. She was receiving medical therapy for both diseases. 4 years ago she had the history of simple hysterectomy secondary to abnormal uterine bleeding. Pathology report showed only simple endometrial hyperplasia without atypia.

Physical examination showed respiratory rate = 16/min, pulse rate = 80/min, blood pressure = 160/100 and normal temperature.

Physical examination of the heart and head and neck examination were unremarkable. Lung examination showed decreased breathing sound in both lungs. Laboratory examination was normal.

Computed tomography scan of the chest showed multiple different-sized nodules in both lungs, the largest measuring 30 mm × 23 mm [Figure 1] abdominal and pelvic sonography was normal, including ovaries, pancreas and liver all of which were unremarkable.
Figure 1: Computed tomography scan of the chest shows multiple varying size nodules in right and left lungs

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The patient scheduled for operation and posterolateral thoracotomy incision adjacent to 5 th intercostal space was performed. Pleural cavity was entered. Several small and large bullae like masses were detected in the right lung which was resected. There was no lymphadenopathy in the chest and mediastinum.

The specimen received in the pathology department consisted of several varying-sized thin-walled cystic lesions filled with moderately viscous material [Figure 2].
Figure 2: Gross pathology of the multiple thin-walled cystic lesions

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Microscopic sections of the cysts showed mucinous epithelium with no atypia [Figure 3]a and b]. Beneath the epithelium the stroma consisted of bland spindle shaped cells which were strongly positive with estrogen receptor antibody [Figure 3]c.
Figure 3: (a and b) Section from the cyst wall shows mucinous epithelium with underlying ovarian type stroma (a) (H and E, 100), (b) (H and E, 250) (c) estrogen receptor positivity in the subepithelial stroma

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Other immunohistochemical findings showed positive epithelium with AE1/AE3 and cytokeratin 7 (CK7), as well as negative epithelium with CK20 and CK19. Ovarian type stroma was positive with vimentin and estrogen receptor.

The diagnosis of mucinous cystic neoplasm with ovarian type stroma was made for the patient. Our plan was to perform another surgery but she didn't accept another operation. She is under observation and in good health, with no complaint of respiratory problem, although the imagings still show some small cystic structures [Figure 4]
Figure 4: Follow-up chest X-ray shows multiple varying size small cysts in both lungs

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Most of the previously reported cases have been asymptomatic and detected during routine medical examination. [2] These cystic tumors are most commonly unilocular and single and rarely cause compromising respiratory function. [3] Our present case has been unique, presented with cough and dyspnea, because it was multiple in both lungs. The usual presentation of the rare published cases in the English literature has been single, round, well-circumscribed and cystic mass at the peripheral lung field. [4]

World health organization classification recognizes mucinous cystic neoplasm as "a localized cystic mass filled with mucin and surrounded by a fibrous wall lined by well-differentiated columnar mucinous epithelium." It seems that there are some exceptions in this definition, because our case presented with multiple thin walled cystic spaces lined by mucinous epithelium and also with ovarian type stroma in their walls. This presentation has been reported in the liver and pancreas, [3] but has not been reported in the lung, meanwhile in our case, the ovarian type stroma was strongly positive with estrogen and progesterone receptor antibody. The proposed theory for this occurrence is the misplaced germ cells in the lung or other tissues. [4] Cases of cystadenoma with ovarian type stroma in the liver and pancreas are exclusively reported in middle-aged women and have two essential tumor components: a cyst lining of columnar to cuboidal mucin secreting epithelium and moderate to dense cellular stroma composed of spindle cells. [5] However, this occurrence has not been reported in the lung.

The best treatment of this tumor appears to be complete surgical excision by lobectomy. The role of adjuvant therapy is unclear. [4]

These types of cysts are said to have a good prognosis after complete resection and recurrence has rarely been reported. [3] However, borderline and malignant counterpart of pulmonary mucinous neoplasm has also been reported. [1]

As a conclusion, to the best of our knowledge, this case is the first report of a multifocal primary pulmonary mucinous cystic neoplasm with ovarian type stroma in the English literature.

 
   References Top

1.Gao ZH, Urbanski SJ. The spectrum of pulmonary mucinous cystic neoplasia: A clinicopathologic and immunohistochemical study of ten cases and review of literature. Am J Clin Pathol 2005;124:62-70.  Back to cited text no. 1
    
2.Guimaraes AR, Wain JC, Mark EJ, Wittram C. Mucinous cystadenoma of the lung. AJR Am J Roentgenol 2004;183:282.  Back to cited text no. 2
    
3.Igai H, Okumura N, Ohata K, Matsuoka T, Kameyama K, Nakagawa T. Pediculate mucinous cystadenoma difficult to differentiate from pleural tumor. Ann Thorac Surg 2008;85:1807-9.  Back to cited text no. 3
    
4.Haruki T, Nakamura H, Taniguchi Y, Miwa K, Adachi Y, Fujioka S. Pulmonary mucinous cystadenoma: A rare benign tumor of the lung. Gen Thorac Cardiovasc Surg 2010;58:287-90.  Back to cited text no. 4
    
5.Geramizadeh B, Asadian M, Hourang MH. Hepatobiliary cystadenoma mimicking liver hydatid cyst. Arch Iran Med 2010;13:556-8.  Back to cited text no. 5
    

Top
Correspondence Address:
Bita Geramizadeh
Department of Pathology, Shiraz University of Medical Sciences, Shiraz
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.130910

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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