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Year : 2016  |  Volume : 59  |  Issue : 1  |  Page : 90-92
Synchronous existence of granular cell tumor and small cell carcinoma of lung: An unusual entity

1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pulmonary Medicine and Sleep Disorder, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication9-Mar-2016


Granular cell tumor (GCT) is a rare benign mesenchymal tumor that uncommonly occurs in the lung and tracheobronchial tree. Small cell carcinoma of lung is a centrally located malignant neoplasm that commonly occurs in elderly smokers. Concomitant existence of both the neoplasm in lung is extremely rare with only one reported case in the literature. Few rare combinations of GCT with other primary bronchogenic carcinomas have also been reported. Clinical symptoms depend upon the site and size of the tumor. Definitive diagnosis is by histopathological and proper immunohistochemical analysis. Identification of this entity is important as treatment requires individual therapy protocols that depend on the presence of metastasis, location of the tumors, and type of bronchogenic carcinoma.

Keywords: Granular cell tumor, lung, small cell carcinoma, synchronous tumor

How to cite this article:
Nath D, Gupta A, Arava S, Jain D, Madan K. Synchronous existence of granular cell tumor and small cell carcinoma of lung: An unusual entity . Indian J Pathol Microbiol 2016;59:90-2

How to cite this URL:
Nath D, Gupta A, Arava S, Jain D, Madan K. Synchronous existence of granular cell tumor and small cell carcinoma of lung: An unusual entity . Indian J Pathol Microbiol [serial online] 2016 [cited 2021 Oct 28];59:90-2. Available from: https://www.ijpmonline.org/text.asp?2016/59/1/90/178239

   Introduction Top

Granular cell tumor (GCT) is a rare benign mesenchymal tumor of Schwann cell origin commonly seen in the tongue, skin and breast. Occurrence in the endobronchial location is extremely rare. [1] Small cell carcinoma of the lung comprises 10-20% of all lung cancer with a central location and male preponderance. It has a strong association with smoking. [2] Concomitant existence of pulmonary GCTs and bronchogenic carcinomas are extremely rare with few reported cases in the literature. Till now there is only a single case report of GCT with small cell carcinoma lung has been described. The present case is highlighted because of its rarity with important clinical significance and different treatment aspects.

   Case report Top

A 55-year-old female with a smoking index of 500 presented in the pulmonary outpatient department with chief complaints of chest pain, shortness of breath and facial puffiness for 2 weeks duration. Patient was a known case of diabetes and essential hypertension for last 7 years and was on regular medications for the same. On general physical examination, dilated veins were visible in the neck and upper chest region suggesting superior vena caval obstruction. Examination of the respiratory system revealed slightly reduced breath sound intensity in the right infraclavicular area. Rest of the systemic examination was unremarkable. Posteroanterior chest radiograph showed widening of the right paratracheal area. Contrast enhanced computed tomography scan [Figure 1]a] of the thorax demonstrated a right paratracheal mass showing internal heterogenous areas with speculated margins. There was an extension of the mass into the subcarinal area. Flexible bronchoscopic examination [Figure 1]b] showed a widened carina and a bulge (extrinsic compression) in the intermediate bronchus with areas of mucosal nodularity. Based on above clinical and radiological findings, a diagnosis of carcinoma lung with bronchial involvement was made. Bronchial washings, transbronchial needle aspiration from the subcarinal lymph node and bronchial biopsy from the bronchial mucosal nodularity in the intermediate bronchus were performed in the same setting.
Figure 1: (a) Contrast enhanced computed tomography scan of the thorax (axial section, mediastinal window) demonstrating a right paratracheal mass with spiculated margins. (b) Flexible bronchoscopy shows mucosal nodularity in the intermediate bronchus

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   Histopathology Top

Bronchial wash and fine needle aspiration cytology both showed cytomorphological features of a small cell carcinoma with tumor cells showing positivity for cytokeratin and synaptophysin [Figure 2]a and b]. Bronchial biopsy from endobronchial mucosal nodularity [Figure 2]c and d] showed tumor cells arranged in clusters as well as in small groups. The individual tumor cells were polygonal in shape with round nuclei, vesicular chromatin, and abundant granular eosinophilic cytoplasm. On immunohistochemistry, these tumor cells were immunopositive for S100, CD68 and neuron-specific enolase (NSE). They were immunonegative for smooth muscle actin (SMA) and p53. Mib-1 labeling index was <1%. The above histomorphological and immuhistochemical features confirmed the diagnosis of benign GCT of the tracheobronchial tree.
Figure 2: Fine needle aspiration cytology smears of small cell carcinoma (a) shows extensive crush artifact with high N:C ratio and powdery nuclear chromatin with (b) synaptophysin immunopositivity in the tumor cells. (c) Histology of the granular cell tumor shows polygonal cells arranged in clusters and in small groups with round nuclei, vesicular chromatin and abundant eosinophilic granular cytoplasm with (d) immunopositivity for neuron - specific enolase and nuclear positivity for S100 protein (inset with arrow)

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   Discussion Top

Granular cell tumors are rare mesenchymal tumor first described by Abni Kossoft in 1926. The diagnostic criteria as proposed by Fanburg-Smith et al. in 1998 are used to divide GCTs into three categories-benign, atypical and malignant-based on six histological criteria. These include - (1) necrosis, (2) spindling, (3) vesicular nuclei with large nucleoli, (4) increased mitotic activity, (5) high nuclear-cytoplasmic ratio and (6) pleomorphism. Tumors that exhibited minimum three of these criteria were characterized as malignant. [3] Occurrences of these tumors in endobronchial location are rare and till now only 80 isolated cases have been described in the literature. [1] Malignant GCT of the tracheobronchial tree is extremely rare with only one reported case with distant metastasis, however, most of the pulmonary GCT behave in a benign fashion. [4] An extensive search of the literature revealed six cases of benign pulmonary GCTs coexisting with bronchogenic carcinoma. This included three adenocarcinomas, two squamous cell carcinomas, and one small cell carcinoma. [5] These tumors tend to occur classically in the middle-aged patients, and the mean age of presentation is in the third decade of life (36-37 years) with equal sex incidence. Tracheal tumors are more prevalent in women, and bronchial tumors are seen equally in males and females. [5] The etiology of GCT is little known, however, association with cigarette smoking is frequently noted. The index case has also the history of cigarette smoking but because of the small number of patients and insufficient database, the association between GCT and smoking is not clearly established. Small cell carcinoma of the lung is however associated with smoking in 85% of cases. Pulmonary GCTs are usually seen in the lower trachea and central bronchi down to the segmental level. They have a tendency to occur at bifurcation site. [6] The GCTs in our case had a central bronchial location. Pulmonary GCTs are usually solitary but can be multiple in 7-25% of cases. [6] In our case, GCT was presented as a multiple nodular submucosal lesion. Pulmonary GCT is also reported in association with other diseases such as HIV infection, sarcoidosis, cerebral arteriovenous malformation and pulmonary chondroid hamartoma. [7]

Grossly, these tumors appear well circumscribed and on microscopy they are composed of cells with abundant eosinophilic granular cytoplasm with a small and uniform nucleus. On immunohistochemical study, the tumor cells are immunopositive for S100 protein, NSE, vimentin, SMA and CD68, while cells of small cell carcinoma shows immunoreactivity for synaptophysin, chromogranin and cytokeratin with proliferative index of more than 90%.

The clinical presentations of the patients vary depending upon the size, and location of the tumor. Most of the patients presented with hemoptysis, dyspnea, and pneumonia while others are asymptomatic and diagnosed incidentally. The bronchoscopic finding in symptomatic patients is generally a solitary lesion in the trachea or in the bronchus. [6] On imaging studies, these tumors can present as bronchial obstruction, coin lesion or as a hilar mass. [8] As these lesions are located in the proximal airway, bronchoscopy and biopsy often provide a definitive diagnosis. Endoscopically these lesions are difficult to excise because of their local infiltration into the peribronchial tissue. [7] There is a high recurrence rate when these tumors are removed either endoscopically or by laser therapy. However, a recent article reported successful use of Nd: YAG laser to remove a small GCT in an elderly patient, with no recurrence. [9] It is therefore been suggested that lesions of 8-10 mm or larger should be removed surgically. Pneumonectomy and lobectomy should be reserved for those tumors associated with profound destruction of the distal lung tissue. Other treatment modalities include cryotherapy, laser or electrocautery. There have been case report of patients with GCT who did not receive any treatment, but none of them died during their follow-up. [10] Coexistence of GCT and bronchogenic carcinoma requires individual therapy protocol that depends on the presence of metastases, location of the tumors, and type of bronchogenic carcinoma.

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   References Top

Dail HD, Hammer SP, editors. Granular cell myoblastoma. In: Pulmonary Pathology. 2 nd Edition. New York. Springer-Verlag; 1994. p. 1355-7.  Back to cited text no. 1
Diggs CH, Engeler JE Jr, Prendergast EJ, Kramer K. Small cell carcinoma of the lung. Treatment in the community. Cancer 1992;69:2075-83.  Back to cited text no. 2
Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: Diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol 1998;22:779-94.  Back to cited text no. 3
Callejo SA, Kronish JW, Decker SJ, Cohen GR, Rosa RH Jr. Malignant granular cell tumor metastatic to the orbit. Ophthalmology 2000;107:550-4.  Back to cited text no. 4
Gabriel JB Jr, Thomas L, Mendoza CB, Chauhan PM. Granular cell tumor of the bronchus coexisting with a bronchogenic adenocarcinoma: A case report. J Surg Oncol 1983;24:103-6.  Back to cited text no. 5
Thomas de Montpréville V, Dulmet EM. Granular cell tumours of the lower respiratory tract. Histopathology 1995;27:257-62.  Back to cited text no. 6
Ganti S, Marino W. Granular cell myoblastoma in an HIV positive patient. N Y State J Med 1991;91:265-6.  Back to cited text no. 7
Szczepulska-Wójcik E, Langfort R, Kupis W, Giedronowicz D, Wiatr E, Quandil N, et al. Granular cell tumor - A rare, benign respiratory tract neoplasm in the material of the Institute of Tuberculosis and Lung Diseases. Pneumonol Alergol Pol 2004;72:187-91.  Back to cited text no. 8
Epstein LJ, Mohsenifar Z. Use of Nd: YAG laser in endobronchial granular cell myoblastoma. Chest 1993;104:958-60.  Back to cited text no. 9
Van der Maten J, Blaauwgeers JL, Sutedja TG, Kwa HB, Postmus PE, Wagenaar SS. Granular cell tumors of the tracheobronchial tree. J Thorac Cardiovasc Surg 2003;126:740-3.  Back to cited text no. 10

Correspondence Address:
Sudheer Arava
Assistant Professor, Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.178239

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