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Department of Radiology
Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Case of the Month
Case of the Month
Spindle cell carcinoma of the lung
Spindle cell carcinoma of the lung
Contributed by: Prakash Rana
Contributed by: Prakash Rana
Introduction:
Introduction:
Spindle cell carcinoma (SpCC) is a rare subgroup of sarcomatoid carcinoma ,a small subset of non–small cell lung cancer (NSCLC). Sarcomatoid carcinoma (SC) of the lung are rare, its incidence is 0.3–1.3% of all lung malignancies (4). It is a heterogeneous group of tumors without any definite diagnostic criteria (5).There are only 13 reported cases in literature, only three of which had a survival of more than 11 months. Because of the rarity of disease, there is no standard strategy for the treatment of SC including SpCC.
Spindle cell carcinoma (SpCC) is a rare subgroup of sarcomatoid carcinoma ,a small subset of non–small cell lung cancer (NSCLC). Sarcomatoid carcinoma (SC) of the lung are rare, its incidence is 0.3–1.3% of all lung malignancies (4). It is a heterogeneous group of tumors without any definite diagnostic criteria (5).There are only 13 reported cases in literature, only three of which had a survival of more than 11 months. Because of the rarity of disease, there is no standard strategy for the treatment of SC including SpCC.
We present a case of a 20-year-old woman who was diagnosed with a lung mass - later proven to be SpCC, We believe this case will be a useful contribution to the literature as this type of cancer is rarely seen and even less frequently reported.
We present a case of a 20-year-old woman who was diagnosed with a lung mass - later proven to be SpCC, We believe this case will be a useful contribution to the literature as this type of cancer is rarely seen and even less frequently reported.
Clinical Profile:
Clinical Profile:
A 20 year old girl presented with the complaints of cough since two weeks and blood tinged sputum since one week . The patient denied shortness of breath, chest pain, productive cough, weight loss, night sweats, or loss of appetite. The patient was anemic (Hb – 6.8gm/dl)
A 20 year old girl presented with the complaints of cough since two weeks and blood tinged sputum since one week . The patient denied shortness of breath, chest pain, productive cough, weight loss, night sweats, or loss of appetite. The patient was anemic (Hb – 6.8gm/dl)
Radiological findings:
Radiological findings:
The chest Radiograph showed a well defined, lobulated soft tissue opacity in left suprahilar region measuring approximately 5 x 4cm abutting the hilum .The margins of the opacity are lobulated and well defined. Inferomedially it is seen to blend with the left hilum. No calcification or cystic lucencies are in the lesion. Bones are normal.(Fig 1A)
The chest Radiograph showed a well defined, lobulated soft tissue opacity in left suprahilar region measuring approximately 5 x 4cm abutting the hilum .The margins of the opacity are lobulated and well defined. Inferomedially it is seen to blend with the left hilum. No calcification or cystic lucencies are in the lesion. Bones are normal.(Fig 1A)
The lateral chest radiograph confirmed the lesion to be in anterior and middle mediastinum.(Fig 1B).
The lateral chest radiograph confirmed the lesion to be in anterior and middle mediastinum.(Fig 1B).
Plain and contrast enhanced CT scan of thorax showed a heterogeneously, intensely enhancing soft tissue mass, 7x5.5x6 cm in size in relation to the the left upper lobe with few non enhancing areas within. There is compression of left upper lobe bronchus with collapse of the anterior segment of left upper lobe. There were multiple, enlarged, homogeneously enhancing lymph nodes in the prevascular, pretracheal and paratracheal regions.The fat plane between the mass and left pulmonary artery was obliterated (Fig 3A , 3B) There were few hyperdense areas on plain scan suggestive of hemorrhage (Fig 4).
Plain and contrast enhanced CT scan of thorax showed a heterogeneously, intensely enhancing soft tissue mass, 7x5.5x6 cm in size in relation to the the left upper lobe with few non enhancing areas within. There is compression of left upper lobe bronchus with collapse of the anterior segment of left upper lobe. There were multiple, enlarged, homogeneously enhancing lymph nodes in the prevascular, pretracheal and paratracheal regions.The fat plane between the mass and left pulmonary artery was obliterated (Fig 3A , 3B) There were few hyperdense areas on plain scan suggestive of hemorrhage (Fig 4).
Radiological diagnosis:
Radiological diagnosis:
Bronchogenic carcinoma
Bronchogenic carcinoma
Bronchoscopy revealed a pearly, whitish endoluminal growth obstructing the left anterior upper lobar bronchus.The mass was friable and bled on touch.Biopsy of the mass was done. (Fig 5)
Bronchoscopy revealed a pearly, whitish endoluminal growth obstructing the left anterior upper lobar bronchus.The mass was friable and bled on touch.Biopsy of the mass was done. (Fig 5)
Post biopsy, the patient developed hemoptysis and difficulty in breathing and was shifted to non invasive ventilation.
Post biopsy, the patient developed hemoptysis and difficulty in breathing and was shifted to non invasive ventilation.
Repeat Chest radiograph showed complete opacification of left hemithorax secondary to hemorrhage obstructing the left lobar bronchus with collapse.(Fig 6)
Repeat Chest radiograph showed complete opacification of left hemithorax secondary to hemorrhage obstructing the left lobar bronchus with collapse.(Fig 6)
Repeat plain CT scan of thorax showed the heterogenous mass with hyperdense areas within in the left upper lobe and near complete collapse of left lung.(Figure 7A,B).There are multiple nodular opacities in the right lung sparing the apex and right middle lobe suggestive of aspiration.(Fig 7C).
Repeat plain CT scan of thorax showed the heterogenous mass with hyperdense areas within in the left upper lobe and near complete collapse of left lung.(Figure 7A,B).There are multiple nodular opacities in the right lung sparing the apex and right middle lobe suggestive of aspiration.(Fig 7C).
Pathological diagnosis:
Pathological diagnosis:
Spindle cell carcinoma
Spindle cell carcinoma
Biopsy of the lesion showed blood clot admixed with fibro myxoid fragments and multiple spindle cells suggestive of spindle cell lesion (Figure 8A,B,C,D).
Biopsy of the lesion showed blood clot admixed with fibro myxoid fragments and multiple spindle cells suggestive of spindle cell lesion (Figure 8A,B,C,D).
Sequential radiograph ten days later showed resolution of aspiration and collapse of the lung.
Sequential radiograph ten days later showed resolution of aspiration and collapse of the lung.
Figure 1 A well defined lobulated soft tissue mass in left supra hilar region.
Figure 2 Lateral chest radiograph.
Figure 3A a heterogenously intensely enhancing mass in left upper lobe with few non enhancing areas within
Figure 3B Coronal reformated image
Figure 4 Few hyperdense foci suggestive of hemorrhage.
Figure 5 Pearly white lesion compressing the left upper lobe bronchus.
Figure 6 Near complete opacification of left hemithorax suggestive of collapse.
Figure 7A Mass with hyperdense areas within
Figure 7B Near complete collapse of left lung.
Figure 7C Nodular opacities in dependent areas post biopsy representing aspiration. Nodular opacities in dependent areas post biopsy representing aspiration.
Figure 8A 4X Mucigenic lining.
Figure 8B 4X Blood clots admixed with spindle cells.
Figure 8C 10X.
Figure 8D 100X Multiple spindle cells are present.
Figure 9 Complete resolution of left lung collapse.
Discussion:
Discussion:
Sarcomatoid carcinoma (SC) of the lung are rare, its incidence is estimated in 0.3–1.3% of all lung malignancies and comprises a small subset of non–small cell lung cancer (NSCLC).
Sarcomatoid carcinoma (SC) of the lung are rare, its incidence is estimated in 0.3–1.3% of all lung malignancies and comprises a small subset of non–small cell lung cancer (NSCLC).
Based on histological features, sarcomatoid carcinoma ,is classified into five major types by the World Health Organization (WHO), into pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma.(4)Spindle cell is a rare histological subtype. (5)
Based on histological features, sarcomatoid carcinoma ,is classified into five major types by the World Health Organization (WHO), into pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma.(4)Spindle cell is a rare histological subtype. (5)
Spindle cells are of mesenchymal origin, and form the body's connective tissue, fat, muscle, bone, cartilage and blood vessels. The name 'spindle cell' refers to the shape of the cell on cytology and histology.
Spindle cells are of mesenchymal origin, and form the body's connective tissue, fat, muscle, bone, cartilage and blood vessels. The name 'spindle cell' refers to the shape of the cell on cytology and histology.
Pulmonary spindle cell carcinoma (SpCC) is derived from epithelium, but exhibits characteristics of mesenchymal differentiation. SpCC occurs in the oral cavity, larynx, breast, kidney, uterus, conjunctiva, prostate, other organs, and occasionally in the lungs.(6,7,8)
Pulmonary spindle cell carcinoma (SpCC) is derived from epithelium, but exhibits characteristics of mesenchymal differentiation. SpCC occurs in the oral cavity, larynx, breast, kidney, uterus, conjunctiva, prostate, other organs, and occasionally in the lungs.(6,7,8)
The exact cause of SpCC of Lung is unknown. However, it is believed that there are certain factors contributing to the condition, which include smoking, exposure to radon, and prolonged exposure to asbestos and other harmful chemicals (arsenic, chromium, nickel, and tar)
The exact cause of SpCC of Lung is unknown. However, it is believed that there are certain factors contributing to the condition, which include smoking, exposure to radon, and prolonged exposure to asbestos and other harmful chemicals (arsenic, chromium, nickel, and tar)
Radiographically, SpCC lesions have been reported to have low-density regions in the center on CT scan, (9) SpCC is diagnosed on biopsy by the appearance of heavily mitotic malignant spindle cells with hyperchromic nuclei and an absence of pleomorphic or giant cells.
Radiographically, SpCC lesions have been reported to have low-density regions in the center on CT scan, (9) SpCC is diagnosed on biopsy by the appearance of heavily mitotic malignant spindle cells with hyperchromic nuclei and an absence of pleomorphic or giant cells.
Immunohistochemistry (IHC) in SpCC has been described to show strong positive reactions for cytokeratin and vimentin and negative reactions for desmin, S100 protein, α-smooth muscle actin, and CD34(10).P63 can be weakly positive, which has a fundamental role in the epithelial development and plays a role in a number of tumors of epithelial origin (11). Seventy-five percent of the lung sarcomatoid cancers (pleomorphic, spindle cell, giant cell) are positive for PD-L1. PD-L1 expression confers a poor prognosis but, on the other hand, also makes the patient a candidate for targeted immunotherapy therapy(12)
Immunohistochemistry (IHC) in SpCC has been described to show strong positive reactions for cytokeratin and vimentin and negative reactions for desmin, S100 protein, α-smooth muscle actin, and CD34(10).P63 can be weakly positive, which has a fundamental role in the epithelial development and plays a role in a number of tumors of epithelial origin (11). Seventy-five percent of the lung sarcomatoid cancers (pleomorphic, spindle cell, giant cell) are positive for PD-L1. PD-L1 expression confers a poor prognosis but, on the other hand, also makes the patient a candidate for targeted immunotherapy therapy(12)
Pulmonary spindle cell carcinoma should be distinguished from other spindle cell tumors as follows:
Pulmonary spindle cell carcinoma should be distinguished from other spindle cell tumors as follows:
1. Inflammatory myofibroblastic tumor: IMT is generally more common in children and young people. The basic structures of IMT are composed of inflammatory cells and myofibroblasts (spindle-like or epithelioid). The interstitial space may exhibit edematous, mucous, fibrous, or hyaline changes. Mitoses are usually rare (<2/10 HPFs). Immunohistochemical features include different degrees of actin (SMA), ALK, and desmin positivity. ALK gene rearrangement is a feature of IMT.
1. Inflammatory myofibroblastic tumor: IMT is generally more common in children and young people. The basic structures of IMT are composed of inflammatory cells and myofibroblasts (spindle-like or epithelioid). The interstitial space may exhibit edematous, mucous, fibrous, or hyaline changes. Mitoses are usually rare (<2/10 HPFs). Immunohistochemical features include different degrees of actin (SMA), ALK, and desmin positivity. ALK gene rearrangement is a feature of IMT.
2. Malignant solitary fibrous tumor: The diameter of a malignant solitary fibrous tumor is often >10 cm, and it often contains dense areas of tumor cells with obvious sarcomatoid and atypical features. The nuclei are pleomorphic with mitosis >4/10 HPF. These tumors are STAT6-positive and EMA-negative
2. Malignant solitary fibrous tumor: The diameter of a malignant solitary fibrous tumor is often >10 cm, and it often contains dense areas of tumor cells with obvious sarcomatoid and atypical features. The nuclei are pleomorphic with mitosis >4/10 HPF. These tumors are STAT6-positive and EMA-negative
3. Synovial sarcoma: In this kind of tumor, the spindle cells are homogenous and relatively small with an oval-shaped and lightly stained nucleus, and the nucleolus is not obvious. The cytoplasm is sparse and the cell boundaries are unclear. Most cells are arranged in short bundles. It has a characteristic t (X;18)(p11;q11) translocation that produces an SYT-SSX fusion gene. Immunohistochemical features include expression of EMA and broad-spectrum CK, CD99, and TLE1.
3. Synovial sarcoma: In this kind of tumor, the spindle cells are homogenous and relatively small with an oval-shaped and lightly stained nucleus, and the nucleolus is not obvious. The cytoplasm is sparse and the cell boundaries are unclear. Most cells are arranged in short bundles. It has a characteristic t (X;18)(p11;q11) translocation that produces an SYT-SSX fusion gene. Immunohistochemical features include expression of EMA and broad-spectrum CK, CD99, and TLE1.
4. Sarcomatoid mesothelioma: Clinical manifestations of this tumor often include pleural effusions, pleural nodules, and pleural thickening. Microscopic features include a bundle or cluster arrangement of the spindle cells, similar to fibrosarcoma tumor cells. D2–40 and WT1 are positive.
4. Sarcomatoid mesothelioma: Clinical manifestations of this tumor often include pleural effusions, pleural nodules, and pleural thickening. Microscopic features include a bundle or cluster arrangement of the spindle cells, similar to fibrosarcoma tumor cells. D2–40 and WT1 are positive.
Conclusion:
Conclusion:
SpCC contains both histologic characteristics of epithelial and mesenchymal tumor, little is known about its presentation, diagnostics and treatment. It typically presents with nonspecific clinical symptoms and image findings similar to other lung cancers making histopathology and Immunohistochemistry paramount for a definitive diagnosis. The literature on the treatment and disease progression is still very limited, and there is a need for publishing long-term follow-up of such patients.
SpCC contains both histologic characteristics of epithelial and mesenchymal tumor, little is known about its presentation, diagnostics and treatment. It typically presents with nonspecific clinical symptoms and image findings similar to other lung cancers making histopathology and Immunohistochemistry paramount for a definitive diagnosis. The literature on the treatment and disease progression is still very limited, and there is a need for publishing long-term follow-up of such patients.
References:
References:
1 Gupta R, Singh S, Hedau S, et al. Spindle cell carcinoma of head and neck: an immunohistochemical and molecular approach to its pathogenesis. J Clin Pathol 2007;60:472–5.doi:10.1136/jcp.2005.033589
1 Gupta R, Singh S, Hedau S, et al. Spindle cell carcinoma of head and neck: an immunohistochemical and molecular approach to its pathogenesis. J Clin Pathol 2007;60:472–5.doi:10.1136/jcp.2005.033589
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2.Feng L,Cai D,Muhetaer A, et al. Spindle cell carcinoma: the general demographics, basic clinico-pathologic characteristics, treatment, outcome and prognostic factors. Oncotarget 2017;8:43228–36.doi:10.18632/oncotarget.18017
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3↵Kida J,Kanaji N,Kishi S, et al. An autopsy case of rapidly progressing spindle cell carcinoma of the lung accompanied with intratumor hemorrhage. Am J Case Rep 2015;16:805–10.doi:10.12659/AJCR.894443
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Acknowledgement:
Acknowledgement:
We are grateful to the Department of Pathology at our institution for providing the histology images and their description
We are grateful to the Department of Pathology at our institution for providing the histology images and their description
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