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  Table of Contents    
CASE REPORT  
Year : 2016  |  Volume : 59  |  Issue : 1  |  Page : 87-89
Primary endobronchial myxoid leiomyoma in a child: An unusual case report and review of literature


1 Department of Pathology, SRL Diagnostics, Fortis Hospital, New Delhi, India
2 Department of Pathology, School of Medical Sciences and Research, Sharda University, Uttar Pradesh, India
3 Department of Thoracic Surgery and Thoracic Surgical Oncology, Fortis Escorts Hospitals, New Delhi, India

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Date of Web Publication9-Mar-2016
 

   Abstract 

Primary leiomyomas are rare benign tumors of the lung and only 25 cases have been documented in children, most of which are endobronchial. Leiomyomas are benign smooth muscle neoplasms, usually diagnosed on morphological characteristics. However, immunohistochemistry plays a crucial role in the lineage differentiation when these are encountered at unusual sites or with unexpected morphological features. We report a case of endobronchial tumor of a 13-year-old male child who presented with a dry cough and hemoptysis. A mass lesion in the right main bronchus was detected by bronchoscopy and contrast-enhanced computed tomography. On histopathological examination of the resected specimen, a diagnosis of myxoid leiomyoma was made. To the best of our knowledge, this is the first case of myxoid leiomyoma to be reported in the respiratory tract. This case also demonstrates the need for a high index of suspicion and the role of immunomarkers in the diagnosis of such challenging cases.

Keywords: Endobronchial, immunohistochemistry, leiomyoma, myxoid

How to cite this article:
Awasthi A, Dubey S, Sabhikhi AK, Bal S. Primary endobronchial myxoid leiomyoma in a child: An unusual case report and review of literature. Indian J Pathol Microbiol 2016;59:87-9

How to cite this URL:
Awasthi A, Dubey S, Sabhikhi AK, Bal S. Primary endobronchial myxoid leiomyoma in a child: An unusual case report and review of literature. Indian J Pathol Microbiol [serial online] 2016 [cited 2019 Dec 8];59:87-9. Available from: http://www.ijpmonline.org/text.asp?2016/59/1/87/174830



   Introduction Top


Benign neoplasms involving the respiratory tract are uncommon and primary leiomyomas are extremely rare. Till date, 25 cases of leiomyomas have been reported in children, most of which are endobronchial. [1],[2],[3],[4],[5],[6],[7],[8],[9] However, myxoid leiomyomas in the respiratory tract have not been documented in the world literature.

In this report, we describe, probably for the 1 st time, a case of primary endobronchial myxoid leiomyoma in a child who presented with nonspecific respiratory symptoms, with a special emphasis on the role of histopathology and immunohistochemistry in reaching a definitive diagnosis in such an unusual case.


   Case report Top


A 13-year-old male child presented with a history of a dry cough not responding to medication, along with hemoptysis of 3 months' duration. On examination, his vital parameters were normal and normal vesicular breath sounds were auscultated bilaterally. Contrast-enhanced computed tomography (CECT) of the thorax showed an intraluminal, mildly enhancing soft tissue mass lesion in the right main bronchus with cut-off sign, accompanied by collapse and consolidation of the upper and middle lobes of the right lung [Figure 1]. Fiberoptic bronchoscopy confirmed a mass lesion in the right main bronchus. Lobectomy of the collapsed right upper lobe was performed.
Figure 1: Contrast - enhanced computed tomography image showing intraluminal, mildly enhancing lesion seen in the right main bronchus with cut - off sign (arrow)

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The lobectomy specimen showed an endobronchial mass measuring 3.5 cm 2.5 cm 2.0 cm bulging into the lumen. The parenchyma and pleura appeared to be uninvolved. Macroscopically, the mass was nodular, well-circumscribed, gray-white, and soft to gelatinous. On microscopy, a mass consisting of intersecting fascicles of spindle-shaped cells with overlying pseudostratified columnar epithelial lining was seen [Figure 2]a]. Individual cells had elongated to spindle-shaped nuclei with tapering ends and scant eosinophilic cytoplasm. The surrounding stroma was loose and myxoid [Figure 2]b]. Congested blood vessels and lymphoplasmacytic infiltrate were seen in the superficial portion of the tumor. No cytonuclear pleomorphism, mitotic activity, or necrosis was discerned. A panel of commercially available immunohistochemical markers was used. The spindle cells were strongly positive for smooth muscle actin, desmin, and vimentin [Figure 3]. Staining for myogenin and CD34 was negative and S100 was weakly positive. The proliferative index was low and Ki-67 value was 1%. On the basis of morphological and immunohistochemical findings, the lesion was diagnosed as a bronchial myxoid leiomyoma.
Figure 2: Histopathological examination of submucosal endobronchial nodule (a) bronchial epithelium with underlying smooth muscle fascicles in myxoid stroma (H and E, ×100) (b) spindle - shaped cells with tapering ends and scant eosinophilic cytoplasm present in myxoid stroma (H and E, ×400)

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Figure 3: Spindle - shaped cells show strong cytoplasmic staining for (a) smooth muscle actin (×400) and (b) desmin (×400)

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   Discussion Top


Bronchial leiomyomas are considered to originate from the smooth muscle layer of the bronchioles, but may arise from ectopic foci of smooth muscle, pulmonary vessels, and primitive mesenchymal cells. [9]

Primary leiomyomas of the lung are very rare, accounting for less than 2% of all benign tumors. The first case was recorded by Forkel in 1910. [9] Till date, a little more than 100 cases of bronchopulmonary leiomyomas have been reported in world literature and only 25 have been documented in children. [1],[2],[3],[4],[5],[6],[7],[8],[9] They predominantly occur in middle-aged persons but the youngest patient reported was a 3-month-old infant. [1] Adults show a female preponderance, but in children, it is more common in males. [6]

Leiomyomas are classified on the basis of their anatomic location. They are more frequently encountered in the distal part of the tracheobronchial tree, with 16% occurring in the trachea, 33% in the bronchus, and 51% in the parenchyma. Nearly all the leiomyomas in the pediatric age group are endobronchial. [1]

Clinical features depend on the location of the tumor, its size, and changes in the lung distal to the mass. Tracheal lesions commonly present with dyspnea and wheezing, mimicking asthma. Endobronchial lesions have symptoms related to bronchial obstruction, such as cough, often with hemoptysis. Wheeze, chest pain, and fever may be present. Secondary obstructive effects upon the distal lung parenchyma may occur such as recurrent pneumonia, bronchiectasis, and finally atelectasis. Over 90% cases of parenchymal leiomyomas are asymptomatic. [1],[9] The current case presented with 3 months history of cough and hemoptysis, not relieved by medication. In an endemic zone such as India, this is likely to be misdiagnosed as tuberculosis, unless there is a high index of suspicion, with thorough investigation and follow-up.

The routine chest X-ray findings may be nonspecific. Almost all the parenchymal leiomyomas present as rounded shadows lacking specific features. In endobronchial leiomyomas, the tumor may be obscured by sequelae of bronchial obstruction such as atelectasis, pneumonic infiltration, and sometimes, localized emphysema, hyperlucency, or mediastinal shift. [1] CT thorax is the best modality to confirm and localize an endobronchial mass. In the present case, X-ray findings were nonspecific but CECT detected an endobronchial mass in the right main bronchus, which was confirmed by bronchoscopy. CECT also revealed collapse and consolidation of the distal lung.

Bronchoscopy is used for definitive diagnosis and therapy. It allows visualization, localization, sampling, and removal of the tumor. Bronchoscopic biopsy could not be performed in this case as the mass was highly vascular.

Macroscopically, leiomyomas present as a smooth, mucosa-covered, yellowish-gray, and broad-based polypoidal tumor. On microscopic examination, tracheal and bronchial leiomyomas are cellular whereas parenchymal tumors are more fibrous and show whorling. [1],[9] Myxoid leiomyoma, a highly uncommon variant of uterine leiomyoma, is a soft and translucent tumor with abundant myxoid material between smooth muscle cells. Typically, the tumor margins are well-circumscribed; cytologic atypia and mitotic figures are absent. [10] In this case, a circumscribed, soft, and mucosa-covered mass consisting of spindle-shaped cells against a pale blue myxoid matrix led to the suspicion of a myxoid benign mesenchymal tumor. The superficial congestion, inflammation, and ulceration probably contributed to hemoptysis.

Bronchial leiomyoma requires differentiation from epithelial and mesenchymal spindle cell tumors and tumor-like conditions. The focal presence of inflammatory cells, along with ulceration, helped to exclude more common conditions such as inflammatory polyp and inflammatory myofibroblastic tumor. The absence of epithelial or other mesenchymal elements was important in eliminating differentials such as hamartoma and establishing the neoplastic nature of the lesion.

In challenging cases like this, immunohistochemistry assumes a major role in diagnosis. The strong positivity of the spindle cells for smooth muscle actin, desmin, and vimentin confirmed the smooth muscle lineage and the negative staining for myogenin and CD34 as well as weak positivity for S100 ruled out the possibility of skeletal muscle, vascular or neural origin.

Another problem faced with smooth muscle tumors is in determining whether the tumor is benign or malignant. Mitotic activity is the main criterion used to assess malignancy. Mitotic figures must be <2/HPF to diagnose myxoid leiomyoma. [10] Our case exhibited low cellularity with the absence of nuclear pleomorphism, mitotic figures, and necrosis. Further confirmation of the benign nature was provided by the low proliferative index.

To diagnose a tumor as a primary leiomyoma, it is essential to exclude the possibility of an extrapulmonary leiomyoma/well-differentiated leiomyosarcoma, generally arising in the uterus and metastasizing to the lung. Ultrasonographic screening for uterine fibroids was redundant, as our patient was a male child. Moreover, metastatic lesions tend to be multiple, have high mitotic activity, and frequently show entrapped pulmonary epithelium. A final diagnosis of primary endobronchial myxoid leiomyoma was therefore made.

Smooth muscle tumors, including those arising in the bronchopulmonary region, have been associated with immunodeficiency states [2] such as acquired immune deficiency syndrome in children. [3],[4],[6] Epstein-Barr virus has also been detected in many such cases. [3],[6] This patient tested negative for human immunodeficiency virus.

To conclude, we would like to emphasize that the use of immunohistochemistry enabled the definitive diagnosis of a hitherto undocumented entity, primary endobronchial myxoid leiomyoma, in a child presenting with nonspecific symptoms.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
White SH, Ibrahim NB, Forrester-Wood CP, Jeyasingham K. Leiomyomas of the lower respiratory tract. Thorax1985;40:306-11.  Back to cited text no. 1
    
2.
User IR, Dogru D, Talim B, Orhan D, Karnak I. Endobronchial, pulmonary and liver leiomyomata in a child with primary immune deficiency. Eur J Pediatr Surg 2010;20:423-5.  Back to cited text no. 2
    
3.
Hatano M, Takada H, Nomura A, Ohga S, Ohshima K, Saeki I, et al. Epstein-Barr virus-associated bronchial leiomyoma in a boy with cellular immunodeficiency. Pediatr Pulmonol 2006;41:371-3.  Back to cited text no. 3
    
4.
Chen W, Theodore J. A benign sessile endobronchial leiomyoma in a 2-year-old girl with human immunodeficiency virus (HIV). Ann Otol Rhinol Laryngol 2002;111:284.  Back to cited text no. 4
[PUBMED]    
5.
Karnak I, Akçören Z, Senocak ME. Endobronchial leiomyoma in children. Eur J Pediatr Surg 2000;10:136-9.  Back to cited text no. 5
    
6.
de Chadarévian JP, Wolk JH, Inniss S, Lischner HW, d'Amore F, Faerber EN, et al. A newly recognized cause of wheezing: AIDS-related bronchial leiomyomas. Pediatr Pulmonol 1997;24:106-10.  Back to cited text no. 6
    
7.
Olgun N, Ozaksoy D, Uçan ES, Türkmen MA, Canda T, Oto O, et al. Paediatric endobronchial leiomyoma mimicking asthma. Respir Med 1995;89:581-2.  Back to cited text no. 7
    
8.
Ozçelik U, Kotiloðlu E, Göçmen A, Senocak ME, Kiper N. Endobronchial leiomyoma: A case report. Thorax1995;50:101-2.  Back to cited text no. 8
    
9.
Miller DR. Benign tumours of lung and tracheobronchial tree. Ann Thorac Surg 1969;8:542-60.  Back to cited text no. 9
[PUBMED]    
10.
Zaloudek CJ, Hendrickson MR, Soslow RA. Mesenchymal tumors of the uterus. In: Kurman RJ, Ellenson LH, Ronnett BM, editors. Blaustein's Pathology of the Female Genital Tract. 6 th ed. New York: Springer; 2011. p. 466-7.  Back to cited text no. 10
    

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Correspondence Address:
Suparna Dubey
H-20 Kailash Colony, New Delhi - 110 048
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.174830

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