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  Table of Contents    
CASE REPORT  
Year : 2015  |  Volume : 58  |  Issue : 3  |  Page : 338-340
Primary pulmonary leiomyosarcoma of lung: An unusual entity with brief review


1 Department of Pathology, AIIMS, New Delhi, India
2 Department of Pulmonary Medicine and Sleep Disorder, AIIMS, New Delhi, India

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Date of Web Publication14-Aug-2015
 

   Abstract 

Primary pulmonary leiomyosarcomas are rare and diagnostically challenging group of neoplasms approximately constituting 0.2-0.5% of all primary lung malignancies. They originate from the smooth muscle cells of the bronchial wall, blood vessels or from the pulmonary interstitium. Here we present a case of 45 year old male with history of chronic cough, breathlessness and chest pain for few months. Clinical and radiological workup showed a left upper lobe lung mass. Endobronchial ultrasound guided (EUS) needle biopsy of the mass on histopathology confirmed leiomyosarcoma. Further through evaluation ruled out the possibility of metastasis. A final diagnosis of primary leiomyosarcoma of lung was made and patient was planned for surgical management.

Keywords: Leiomyosarcoma, primary, endobronchial ultrasound guided needle biopsy

How to cite this article:
Nath D, Arava S, Joshi P, Madan K, Mathur S. Primary pulmonary leiomyosarcoma of lung: An unusual entity with brief review. Indian J Pathol Microbiol 2015;58:338-40

How to cite this URL:
Nath D, Arava S, Joshi P, Madan K, Mathur S. Primary pulmonary leiomyosarcoma of lung: An unusual entity with brief review. Indian J Pathol Microbiol [serial online] 2015 [cited 2020 Jan 25];58:338-40. Available from: http://www.ijpmonline.org/text.asp?2015/58/3/338/162868



   Introduction Top


Primary leiomyosarcomas of the lung are extremely rare tumors arising from the smooth muscles of the pulmonary interstitium, bronchi and blood vessels in the order of decreasing frequency. [1] The majority of the leiomyosarcomas arise from the peribronchial smooth muscle cells present around the hilum and most frequently from the larger bronchi of the left lower lobe. [2] Clinical presentation may vary with symptoms those seen in other primary lung neoplasms or they may be asymptomatic and found incidentally on imaging studies. As the metastatic neoplasms are much more common than the primary, all possible investigations should be undertaken to exclude the extrapulmonary primary. The behavior of this neoplasm varies from indolent to very aggressive depending on the histological grade. Management includes complete resection of the tumor with or without palliative therapy.


   Case Report Top


A 45-year-old male nonsmoker presented with a 4 months history of dry cough, breathless at rest, left sided chest pain and hoarseness of voice. He denied any history of fever, hemoptysis, and loss of weight or other extrapulmonary symptoms. He also denied any history of pulmonary tuberculosis, asthma or chronic obstructive pulmonary disease. He was normotensive and nondiabetic. General physical examination was unremarkable with no pallor, icterus, cyanosis, clubbing or lymphadenopathy. Examination of the respiratory system revealed decreased breath sound in left hemithorax. Other systemic examination was unremarkable. Biochemical investigation and hematological parameters were within normal limits. Three induced sputum samples were negative for acid fast bacilli and malignant cells. Montoux test was nonreactive. Imaging studies of abdominal and pelvic organs were essentially within normal limit. Positron emission tomography/computerized tomographic (PET-CT) examination of the chest revealed a heterogeneously ill-defined soft tissue mass lesion of size 5.3 cm × 6 cm × 6.8 cm encasing and causing narrowing of the left main pulmonary artery. The mass is also extending distally around the left main bronchus and causing compression of the left upper lobe bronchus ([Figure 1]a). Based on the above clinical and radiological findings, a diagnosis of carcinoma lung was made. Fiberoptic bronchoscopy revealed mucosal infiltration into the left main bronchus ([Figure 1]b). For the tissue diagnosis endobronchial ultrasound guided biopsy from the tumor was done. Microscopic examination revealed a malignant spindle cell tumor with cells arranged in intersecting fascicles. The individual tumor cells were oval to spindle in shape with minimal nuclear pleomorphism, blunt nuclear ends and darkly stained irregular nuclear chromatin. Cytoplasm was eosinophilic ([Figure 2]a). Mitotic count ([Figure 2]d) was high (3-4/10 HPF). Necrosis was absent. On immunohistochemistry, ([Figure 2]b and c) the tumor cells were immunopositive for smooth muscle actin (SMA), Desmin, Vimentin and were negative for DOG 1, CD117, S100 and Pancytokeratin. KI-67 labeling index was approximately 40-50% in the highest proliferative area. Based on the above histological and immunohistochemical features, a final diagnosis of pulmonary leiomyosarcoma was made. Detailed workup did not reveal any extrapulmonary primary.
Figure 1: (a) Computerized tomographic scan showing heterogeneously ill-defined soft tissue mass with narrowing of the left pulmonary artery and causing compression of left upper lobe bronchus. (b) Fiberoptic bronchoscopy showing mucosal infiltration in the left bronchus


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Figure 2: (a) Photomicrograph showing spindle cells arranged in fascicles and intersecting with each other (H and E, ×400). (b) Tumor cells are immunoreactive with smooth muscle actin (×400) and (c) desmin (×400). (d) Mib-1 labeling index is high (×400)


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The patient was then referred to a cardio-thoracic department where he underwent left upper lobe pneumonectomy. The resected tumor specimen was solid with grayish-white appearance on cut section. Histopathological examination of the resected tumor also confirmed the diagnosis of leiomyosarcoma with focal areas of necrosis (<50%) and moderate nuclear pleomorphism. The mitotic count of the tumor was approximately 6-7/10 HPF making the diagnosis of conventional leiomyosarcoma of grade II (Federation National des Centers de Lutte le Cancer grading system). Postsurgical history was uneventful, and the patient is on follow up until.


   Discussion Top


Primary pulmonary leiomyosarcoma is rare and is the most common pathological type of sarcoma in the lungs. [3] Till now approximately a total of 127 cases have been reported in the literature. [4] They are mostly found in adults with a male to female ratio of 2.5:1. Few cases have also been reported in children. [4] The common risk factors include radiation therapy, chemotherapy, environmental and occupational exposure. [5] There has been a single report of multiple leiomyosarcoma in lung allograft associated with Epstein-Barr virus infection in an immunocompromised patient. [6]

On histopathology, leiomyosarcoma reveals fascicular proliferation of fusiform cells that show intersections at right angles with areas of high mitosis (Atypical) and necrosis. Other histopathological differentials include Sarcomatoid carcinoma (Both CK and Vimentin positive), Monophasic spindle cell synovial sarcoma (Mic-2 positive), malignant peripheral nerve sheath tumor (S-100 positive), gastrointestinal stromal tumor (CD 117, Dog-1 and CD 34 positive), carcinosarcoma (Ck and vimentin positive), intrapulmonary thymoma (Ck positive with interspersed CD 3 positive cells), inflammatory myofibroblastic tumor (IMT) etc. As IMT is one of the most commonly identified lesions in the lung, it can be differentiated by its characteristic histopathological findings like presence of lymphoplasmacytic cells, cytologically bland spindle-shaped myofibroblasts with variable admixture of collagen. These do not show atypical mitosis or pleomorphism. Hence, a panel of immunohistochemical markers for confirming and to exclude other differentials is necessary for proper diagnosis . As pulmonary leiomyosarcoma resembles carcinoma lung in clinical symptoms, physical signs, and radiological imaging studies, histopathological examination is considered as a gold standard in the diagnosis. The radiological features are nonspecific and vary from collapse, consolidation, and mass lesion with bilateral nodular and patchy infiltrate in the hilar prominence. CT scan may show solid to even a heterogenous mass with low attenuation. Pleural fluid cytology and sputum cytology usually do not detect the tumor as like epithelial tumors. These tumors do not show tendency to exfoliate hence do not yield much diagnostic information.

Most commonly, pulmonary sarcomas are of metastatic in origin. Consequently, it is necessary to rule out metastatic extrapulmonary site before establishing a diagnosis of primary pulmonary leiomyosarcoma. Like any other sarcomas these tumors metastasize via blood stream and very infrequently through the lymphatics. Frozen section examination is of limited use because of the similarities with other spindle cell tumors. [2] Routine diagnostic procedure such as cytological examination of the sputum or bronchial washing or brushings are unrewarding in diagnosis of Primary pulmonary leiomyosarcoma (PPL) in contrast to bronchogenic carcinoma. On review of the literature, most of the cases were diagnosed at autopsy or after surgical resection and few were diagnosed by open lung biopsy. The cytological diagnosis has limitations as interpretation of cells obtained from benign or low-grade malignant neoplasm may be difficult at times. Transthoracic and transbronchial needle biopsy are more likely to provide diagnosis.

Localized unilateral pulmonary lesion with no detectable abnormality of the soft tissue, GI tract and genito-urinary system suggest a primary pulmonary lesion. The bilateral pulmonary lesion usually suggests metastatic malignancy and till date only one case of primary bilateral leiomyosarcoma of lung has been reported. [7] It has been suggested that pulmonary leiomyosarcoma in female patients should never be considered primary neoplasm as there are reports of PPL arising in patients with history of hysterectomy for benign leiomyoma several years before presentation. [8] Lymph node metastasis is an unusual feature of pulmonary leiomyosarcomas although it has been described in several case reports. Distant metastasis to the liver, pleura, skin and adrenal glands are also reported. [9] Surgical resection is the mainstay of therapy for pulmonary leiomyosarcoma and is usually curative for small and well-differentiated sarcoma with a 5 years survival rate of 29-40%. [10] The role of radiotherapy is only palliative and chemotherapy is usually advocated for those having bilateral disease, extrathoracic dissemination and refusal for surgery. The most consistent predictor of long-term survival is the histological grade of the tumor than tumor site (endobronchial/parenchymal) or tumor size. [2] Prognosis is generally poor in tumors measuring more than 10 cm and more than eight mitotic figures per 10 high power fields. Primary pulmonary leiomyosarcomas are very rare clinico-pathological entity that requires early detection, complete diagnostic workup and staging in order to decide proper therapy and to determine prognosis.

 
   References Top

1.
Miller DL, Allen MS. Rare pulmonary neoplasms. Mayo Clin Proc 1993; 68:492-8.  Back to cited text no. 1
    
2.
Attanoos RL, Appleton MA, Gibbs AR. Primary sarcomas of the lung: A clinicopathological and immunohistochemical study of 14 cases. Histopathology 1996;29:29-36.  Back to cited text no. 2
    
3.
Janssen JP, Mulder JJ, Wagenaar SS, Elbers HR, van den Bosch JM. Primary sarcoma of the lung: A clinical study with long-term follow-up. Ann Thorac Surg 1994;58:1151-5.  Back to cited text no. 3
    
4.
Cordes BG, Collins BT, McDonald JW, Khosla A, Salimi Z. Fine needle aspiration biopsy of primary leiomyosarcoma arising from a pulmonary vein. Acta Cytol 1999;43:523-6.  Back to cited text no. 4
[PUBMED]    
5.
Govindan R, Subramanian J. Lung cancer. In: Govindan R, editor. The Washington Manual of Oncology. 2 nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008363-76.  Back to cited text no. 5
    
6.
Somers GR, Tesoriero AA, Hartland E, Robertson CF, Robinson PJ, Venter DJ, et al. Multiple leiomyosarcomas of both donor and recipient origin arising in a heart-lung transplant patient. Am J Surg Pathol 1998;22:1423-8.  Back to cited text no. 6
    
7.
Hicks HG. Bronchogenic leiomyosarcoma; case report with necropsy findings. Dis Chest 1957;32:338-40.  Back to cited text no. 7
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8.
Morgan PG, Ball J. Pulmonary leiomyosarcomas. Br J Dis Chest 1980;74:245-52.  Back to cited text no. 8
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9.
Yellin A, Rosenman Y, Lieberman Y. Review of smooth muscle tumours of the lower respiratory tract. Br J Dis Chest 1984;78:337-51.  Back to cited text no. 9
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10.
Moran CA, Suster S, Abbondanzo SL, Koss MN. Primary leiomyosarcomas of the lung: A clinicopathologic and immunohistochemical study of 18 cases. Mod Pathol 1997;10:121-8.  Back to cited text no. 10
    

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Correspondence Address:
Dr. Sudheer Arava
Department of Pathology, AIIMS, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.162868

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