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Year : 2013  |  Volume : 56  |  Issue : 4  |  Page : 434-436
Immunohistochemical analyses of a case of extralobar pulmonary sequestration with chest pain in an adult

1 Division of Pathology, Matsuyamashimin Hospital, Matsuyama, Ehime - 790 0067, Japan
2 Deparment of Thoracic Surgery, Matsuyamashimin Hospital, Matsuyama, Ehime - 790 0067, Japan
3 Department of Pathology, Kochi Medical School, Kochi University, Kochi, Japan

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Date of Web Publication18-Jan-2014


Computed tomography of a Japanese man in his mid-forties with a complaint of right-side chest pain showed a dome-shaped smooth-surfaced mediastinal mass, which was extirpated. The cut surface was highly hemorrhagic and necrotic and not related to the original pulmonary tissues. Although routine sectioning detected bronchial cartilage, immunohistochemical analyses clearly showed the presence of alveolar type II cells; only the alveolar type II cells located at the periphery of this mass showed positive staining for cytokeratins, thyroid transcription factor 1, surfactant protein A, epithelial membrane antigen and Krebs von den Lungen-6. Thus, these analyses are useful for the detection of pulmonary components, even in severely hemorrhagic and necrotic tissues with marked sequestration. The clinical diagnosis was a rare, adult type of extralobar pulmonary sequestration accompanied by chest pain.

Keywords: Adult type, chest pain, extralobar pulmonary sequestration, immunohistochemistry

How to cite this article:
Ohtsuki Y, Uomoto M, Hachisuka Y, Furihata M. Immunohistochemical analyses of a case of extralobar pulmonary sequestration with chest pain in an adult. Indian J Pathol Microbiol 2013;56:434-6

How to cite this URL:
Ohtsuki Y, Uomoto M, Hachisuka Y, Furihata M. Immunohistochemical analyses of a case of extralobar pulmonary sequestration with chest pain in an adult. Indian J Pathol Microbiol [serial online] 2013 [cited 2021 Oct 19];56:434-6. Available from: https://www.ijpmonline.org/text.asp?2013/56/4/434/125362

   Introduction Top

Both the intralobar and extralobar types of pulmonary sequestration are well-known. [1] The frequency of intralobar cases was demonstrated to be higher than that of extralobar cases, [1] and patients with the intralobar type were generally younger with no clinical symptoms detected during a physical examination. [2],[3] On the other hand, clinical signs such as chest pain [4],[5],[6] and hemothorax [7],[8] were rarely found in some adult cases of the extralobar type.

To obtain clear evidence of sequestration in pulmonary tissues, necrotic and hemorrhagic mediastinal tissues were immunohistochemically examined using antibodies to surfactant protein A (SP-A), thyroid transcription factor 1 (TTF-1), KL-6 and epithelial membrane antigen (EMA). Here, we report a rare case of an adult type of extralobar pulmonary sequestration with chest pain.

   Case Report Top

This was a case report of the patient was a Japanese man in his mid-forties with a complaint of chest pain and a mediastinal tumor (3.5 cm × 1.5 cm), which was extirpated [Figure 1]a . The excised mass was fixed in 10% formalin solution and then embedded in paraffin. Dewaxed sections were stained with hematoxylin and eosin. Immunohistochemical staining was performed as described previously [9],[10] by using a labeled streptavidin-biotin complex 2 kit and horseradish peroxidase (Dako Cytomation, Kyoto, Japan) with diaminobenzidine as the substrate; the antibodies used are listed in [Table 1].
Table 1: Anti bodies used in this study

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Written, informed consent was obtained from the patient and his identity has been strictly protected.

The extirpated tumor was dome-shaped, covered by parietal pleura [Figure 1]a and attached to the parietal wall of the pleural cavity. The cut surface was markedly hemorrhagic and necrotic and an internal structure was not observed on macroscopic examination [Figure 1]b. Routine sectioning showed focally ossified bronchial cartilage as well as dilated blood vessels and a bronchial cavity [Figure 2]a. Bronchial epithelial cells, especially reserve cells and bronchial glands were also detected by routine sectioning; however, they had degenerated. However, epithelial cells were not clearly detected. The vague appearance of alveolar structures in the necrotic areas was observed [Figure 2]b. Beneath the parietal pleura, irregular cellular strands and space-forming cell proliferations were observed [Figure 3]a. The proliferating cells tested positive for cytokeratins (CK) such as AE1/AE3, CK7 [Figure 3]b and CK18 [Figure 3c]. Further, the cells on the inner surface of the irregular space were found to be positive for EMA [Figure 3]d and KL-6 [Figure 3]e. Nuclear staining for TTF-1 [Figure 4]a, arrows] and focal staining for SP-A [Figure 4]b, arrows] were observed. Macrophages showed positive staining for CD68 [Figure 4]c] as well as SP-A, even in the necrotic areas. Macrophages were phagocytized SP-A in their cytoplasm. The amorphous mass observed on the diaphragm during the operation showed organized fibrin clots covered by mesothelial cells, which were positive for calretinin [Figure 5].
Figure 1: Macroscopic findings of the mass (a) aft er fi xati on. Note the dome-shaped mass with a smooth surface. The cut surface of the mass showed massive hemorrhage and necrosis (b)

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Figure 2: In the hematoxylin and eosin (H and E)-stained secti on, severe hemorrhage and necrosis were noted; focal staining of bronchial carti lage (a, arrow) and a cavity (a,*) were observed. Vague, alveolar
space-like lumina were observed in the secti on (H and E, ×20). (b) The subpleural region of the mass (H and E, ×100)

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Figure 3: The epithelial cells at a higher magnifi cati on (a) showing diffuse, positi ve staining for cytokerati n (CK)7 (b), CK18 (c), epithelial membrane anti gen (d) and KL-6 (e). The labeled streptavidin-biotin complex method was used. a-e: ×100

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Figure 4: Epithelial cells showing dense nuclear positi vity for thyroid transcripti on factor 1 (a, arrows) (×400) and surfactant protein A (b, arrows) (×200). Macrophages were positi ve for CD68 (c). Labeled
streptavidin-bioti n complex method (×200)

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Figure 5: Fibrin clots on the diaphragm were covered by calreti nin-positive mesothelial cells. Labeled streptavidin-bioti n complex method, ×200

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These results indicate that the proliferating epithelial cells were alveolar epithelial cells that differentiated into type II cells. Bronchial cartilage and alveolar macrophages were present in the necrotic and hemorrhagic areas. Thus, this case was clinically diagnosed as an adult case of extralobar pulmonary sequestration accompanied by chest pain.

   Discussion Top

Pulmonary sequestration is a rare disease that usually occurs in childhood. Most of the adult patients do not show any symptoms, except for chest pain following torsion, [4] hemorrhage followed by chest pain, [5] infarction followed by chest pain [6] and hemothorax. [7],[8] The number of reported cases of intralobar sequestration is higher than that of extralobar cases (400 vs. 133 in the literature). [1] The frequency of pulmonary sequestration has been reported to be 0.15-6.4%. [1] Usually, clinical symptoms were not observed in adult cases. [2],[3] However, in the present case, the patient complained of chest pain due to the location of the mass in the subparietal space. The mass possibly led to the stimulation of intercostal nerve fibers. The exact cause of his chest pain could not be determined, but hemorrhage and necrotic changes in the parietal pleura were thought to stimulate intercostal nerves. In a few previously reported cases, these changes were thought to have caused chest pain. [4],[5],[6]

To the best of our knowledge, the present study is the first immunohistochemical study for the detection of pulmonary sequestration. In the present case, immunohistochemical analysis using the abovementioned antibodies was found to be useful for the identification of alveolar epithelial cells and alveolar macrophages.

Growing epithelial cells forming alveolar-like spaces were positive for not only TTF-1 and SP-A, but also EMA and KL-6, which are fundamental components on the surface of alveolar epithelial cells. [9],[10] These findings are definite evidence of the growth of alveolar epithelial type II cells in the peripheral region of the mass in extralobar pulmonary sequestration. Moreover, CD68-positive alveolar macrophages have been found in human alveolar spaces.

Immunohistochemical analyses of the degenerated extralobar pulmonary sequestration tissues were useful for the identification of growing pulmonary parenchymal cells.

We reported a rare adult case of extralobar pulmonary sequestration with a clinical symptom of chest pain. The growing alveolar epithelial cells at the periphery of the resected lung.

Tissues were immunohistochemically characterized.

   References Top

1.Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R. Lung sequestration: Report of seven cases and review of 540 published cases. Thorax 1979;34:96-101.  Back to cited text no. 1
2.Gompelmann D, Eberhardt R, Heussel CP, Hoffmann H, Dienemann H, Schuhmann M, et al. Lung sequestration: A rare cause for pulmonary symptoms in adulthood. Respiration 2011;82:445-50.  Back to cited text no. 2
3.Hertzenberg C, Daon E, Kramer J. Intralobar pulmonary sequestration in adults: Three case reports. J Thorac Dis 2012;4:516-9.  Back to cited text no. 3
4.Huang EY, Monforte HL, Shaul DB. Extralobar pulmonary sequestration presenting with torsion. Pediatr Surg Int 2004;20:218-20.  Back to cited text no. 4
5.Chun EJ, Goo JM, Lee HJ, Lee CH, Im JG. Extralobar pulmonary sequestration with hemorrhagic infarction in an adult. J Thorac Imaging 2007;22:166-8.  Back to cited text no. 5
6.Tetsuka K, Endo S, Kanai Y, Yamamoto S. Extralobar pulmonary sequestration presenting as hemothorax. Interact Cardiovasc Thorac Surg 2009;9:547-8.  Back to cited text no. 6
7.Avishai V, Dolev E, Weissberg D, Zajdel L, Priel IE. Extralobar sequestration presenting as massive hemothorax. Chest 1996;109:843-5.  Back to cited text no. 7
8.Pinto Filho DR, Avino AJ, Brandão SL. Extralobar pulmonary sequestration with hemothorax secondary to pulmonary infarction. J Bras Pneumol 2009;35:99-102.  Back to cited text no. 8
9.Ohtsuki Y, Nakanishi N, Fujita J, Yoshinouchi T, Kobayashi M, Ueda N, et al. Immunohistochemical distribution of SP-D, compared with that of SP-A and KL-6, in interstitial pneumonias. Med Mol Morphol 2007;40:163-7.  Back to cited text no. 9
10.Ohtsuki Y, Fujita J, Hachisuka Y, Uomoto M, Okada Y, Yoshinouchi T, et al. Immunohistochemical and immunoelectron microscopic studies of the localization of KL-6 and epithelial membrane antigen (EMA) in presumably normal pulmonary tissue and in interstitial pneumonia. Med Mol Morphol 2007;40:198-202.  Back to cited text no. 10

Correspondence Address:
Yuji Ohtsuki
Division of Pathology, Matsuyamashimin Hospital, Matsuyama, Ehime - 790 0067
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.125362

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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