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LETTER TO EDITOR  
Year : 2014  |  Volume : 57  |  Issue : 1  |  Page : 155-156
Calcifying fibrous pseudotumor of the mediastinum: A rare case report


Department of Pathology, The Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India

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Date of Web Publication17-Apr-2014
 

How to cite this article:
Chauhan KR, Shah HU, Trivedi PP, Shah MJ. Calcifying fibrous pseudotumor of the mediastinum: A rare case report. Indian J Pathol Microbiol 2014;57:155-6

How to cite this URL:
Chauhan KR, Shah HU, Trivedi PP, Shah MJ. Calcifying fibrous pseudotumor of the mediastinum: A rare case report. Indian J Pathol Microbiol [serial online] 2014 [cited 2019 Dec 7];57:155-6. Available from: http://www.ijpmonline.org/text.asp?2014/57/1/155/130936


Sir,

Calcifying fibrous pseudotumor (CFT) is a distinct pathological entity occurring in the soft-tissue of extremities, trunk, axilla, pleura, mediastinum and peritoneum, [1] pathologically characterized by hyalinized collagen, psammomatous or dystrophic calcification and lymphoplasmacytic infiltration. They are clinically benign with an extremely low rate of recurrence and complete surgical resection is known to be the treatment of choice. [2] We report a case of CFT of anterior mediastinum in an 8-year-old boy.

An 8-year-old boy presented with the complaint of chronic cough since 2 years. His chest X-ray showed soft tissue opacity in right middle lobe suggesting segmental collapse. Computed tomography thorax was performed which showed a 35 mm × 58 mm sized soft-tissue lesion in anterior mediastinum near cardio phrenic angle with calcified foci abutting adjacent pericardium and pleura [Figure 1]. Radiological diagnoses of teratoma followed by thymoma and pleuropulmonary blastoma were offered. Ultrasonography of abdomen and pelvis was normal. Serum alpha fetoprotein, beta human chorionic gonadotropin and thyroid function tests were within normal limits. CT guided fine-needle aspiration cytology (FNAC) and biopsy was performed. FNAC was hemorrhagic. Biopsy showed only fibrous tissue with calcified nodules and lymphoid cells with no definite opinion [Figure 2]. Complete surgical excision of the mass through right thoracotomy was performed.
Figure 1: Venous phase axial contrast-enhanced computed tomography showing heterogeneously enhancing soft-tissue mass with few internal calcific foci in anterior mediastinum. Lesion abuts adjacent pericardium and pleura showing preserved intervening fat planes

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Figure 2: Low power view showing calcification, fibrosis and muscle (H and E, 100)

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Grossly, the tumor was well-defined and well-encapsulated measuring 7 cm × 5.5 cm × 3.6 cm having a white firm to hard cut surface with gritty sensation. Areas of hemorrhage, necrosis and cystic change were absent [Figure 3]. Microscopic examination showed dense hyalinized collagenous tissue interspersed with benign appearing spindle cells, psammomatous as well as dystrophic calcifications and a variable lymphoplasmacytic inflammatory infiltrate [Figure 4]. Atypia, necrosis, mitoses were absent. Granuloma was absent. Hence on histopathology, diagnosis of CFT was offered and immunohistochemistry (IHC) was given. On IHC, tumor showed vimentin positivity. Actin, CD34, desmin, anaplastic lymphoma kinase-1 (ALK-1) were negative. After surgery on regular follow up till date, patient is symptom free with no evidence of recurrence.
Figure 3: Gross photograph of calcifying fibrous pseudotumor, wellcircumscribed mass with white cut surface

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Figure 4: (a) Low power view showing hyalinization, dystrophic and psammomatous calcification and spindle cell proliferation (H and E, 100), (b) high power view showing spindle cell proliferation without atypia, inflammatory cells and calcification (H and E, 200)

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CFT is a rare benign fibrous lesion characterized by dense hyalinized collagenous tissue in the sparse spindle cells, psammomatous or dystrophic calcifications and variable quantity of lymphoplasmacytic infiltrates. [2] It has been postulated that CFT may represent a sclerosing end stage of inflammatory myofibroblastic tumor (IMT). [2],[3] The differential diagnoses include thymic epithelial tumor, calcifying granulomas, IMT and amyloidoma of chest wall. [2],[4] These lesions can be differentiated from CFT on the basis of conventional histopathology and IHC. Absence of any well-defined granulomas and a negative congo red stain rules out calcifying granuloma or amyloidoma respectively. IMTs rarely contain calcifications and have a myofibroblastic proliferation varying from hyalinized acellular collagen to florid fibroblastic proliferations simulating sarcoma. [3] IMTs demonstrate diffuse positivity for actin, vimentin, variable positivity for CD34 and focal positivity for factor XIIIa in contrast to CFTs which are diffusely positive for factor XIIIa, vimentin and negative for actin and CD34. [3] ALK-1, one of the distinguishing features of IMT is not common in CFT. [5]

To conclude, CFT is a rare tumor to occur in the anterior mediastinum. Complete excision is the treatment of choice for CFT due to its benign nature.


   Acknowledgment Top


The authors wish to express their thanks to Dr. SN Shukla, the Honorary Director and Dr. R.K. Vyas, The Deputy Director of The Gujarat Cancer & Research Institute to allow us to publish this case report.

 
   References Top

1.Eftekhari F, Ater JL, Ayala AG, Czerniak BA. Case report: Calcifying fibrous pseudotumour of the adrenal gland. Br J Radiol 2001;74:452-4.  Back to cited text no. 1
    
2.Chang JW, Kim JH, Maeng YH. Calcifying fibrous pseudotumor of the anterior mediastinum. Korean J Thorac Cardiovasc Surg 2011;44:318-20.  Back to cited text no. 2
    
3.Hill KA, Gonzalez-Crussi F, Chou PM. Calcifying fibrous pseudotumor versus inflammatory myofibroblastic tumor: A histological and immunohistochemical comparison. Mod Pathol 2001;14:784-90.  Back to cited text no. 3
    
4.Peachell M, Mayo J, Kalloger S, Flint J, English J. Calcifying fibrous pseudotumour of the lung. Thorax 2003;58:1018-9.  Back to cited text no. 4
    
5.Nascimento AF, Ruiz R, Hornick JL, Fletcher CD. Calcifying fibrous 'pseudotumor': Clinicopathologic study of 15 cases and analysis of its relationship to inflammatory myofibroblastic tumor. Int J Surg Pathol 2002;10:189-96.  Back to cited text no. 5
    

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Correspondence Address:
Kriti R Chauhan
WZ 1598 G/1E, Nangal Raya, New Delhi - 110 046
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.130936

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    Figures

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

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