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Plasmacytoma of the trachea: a surprising diagnosis

Dear Editor,

A 68-year-old man presented with a complaint of dyspnea on moderate exertion, and physical examination revealed stridor. The patient reported having previously been treated for chronic obstructive pulmonary disease and adenocarcinoma of the prostate, the latter having been treated with 39 radiotherapy sessions. He was a former smoker with a smoking history of 150 pack-years (3 packs/day for 50 years), having quit 4 years prior. We performed contrast-enhanced computed tomography (CT) of the neck and chest, which showed an expansive, well-defined nodular mass in the distal trachea, near the carina, without enhancement or signs of invasion of the tracheal walls (Figures 1 and 2). Bronchoscopy was requested for tumor resection, and symptom resolution was observed after the resection. The histopathological study identified an outer layer with the of appearance of plasmacytoid cells, sometimes with a central eosinophilic nucleolus—"cartwheel appearance"—and hyaline intracytoplasmic inclusions suggestive of Russell bodies. The immunohistochemical profile demonstrated positivity for CD3, CD20, CD45, CD56, kappa light chain, and CD138 in plasmacytes. In the context of the clinical status and test results, the findings were consistent with solitary extramedullary plasmacytoma.

Figure 1
A: Axial CT scan, without contrast, showing an extensive, well-defined nodular mass in the distal trachea, measuring 2.1 × 1.3 × 1.7 cm, without signs of tracheal wall invasion. B: Coronal CT scan, without contrast, showing an expansive, well-defined nodular mass in the distal trachea, at the level of the carina, without signs of tracheal wall invasion.

Figure 2
A: Sagittal CT scan (ROI: 51 HU), without contrast, showing an expansive, well-defined nodular mass in the distal trachea, without signs of tracheal wall invasion. B: Contrast-enhanced axial CT scan (ROI: 61 HU) showing an expansive nodular mass with no contrast uptake.

Diseases involving the trachea or the main bronchi are not common(11 Barbosa AGJ, Penha D, Zanetti G, et al. Foreign body in the bronchus of a child: the importance of making the correct diagnosis. Radiol Bras. 2016;49:340-2.

2 Ribeiro GMR, Natal MRC, Silva EF, et al. Tracheobronchopathia osteochondroplastica: computed tomography, bronchoscopy and histopathological findings. Radiol Bras. 2016;49:56-7.

3 Barbosa BC, Amorim VB, Ribeiro LFM, et al. Tuberculosis: tracheal involvement. Radiol Bras. 2016;49:410-1.
-44 Franco RM, Guimaraes MD, Moreira BL, et al. Enhancing survival with early surgical resection of endobronchial metastasis in a follow-up of ovarian carcinoma. Radiol Bras. 2015;48:130.). Less common still are tracheal tumors, which account for only 1-2% of all respiratory tract tumors(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.,66 Ferretti GR, Bithigoffer C, Righini CA, et al. Imaging of tumors of the trachea and central bronchi. Radiol Clin North Am. 2009;47:227-41.), affecting mainly the lower third of the tract(77 Olmedo G, Rosenberg M, Fonseca R. Primary tumors of the trachea. Clinicopathologic features and surgical results. Chest. 1982;81:701-6.). Such tumors can be locally invasive(33 Barbosa BC, Amorim VB, Ribeiro LFM, et al. Tuberculosis: tracheal involvement. Radiol Bras. 2016;49:410-1.), adenoid cystic carcinoma and squamous cell carcinoma being the malignant tumors most often affecting the trachea(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.,66 Ferretti GR, Bithigoffer C, Righini CA, et al. Imaging of tumors of the trachea and central bronchi. Radiol Clin North Am. 2009;47:227-41.,88 Reyhan M, Tercan F, Ergin M, et al. Sonographic diagnosis of a tracheal extramedullary plasmacytoma. J Ultrasound Med. 2005;24:1031-4.). The most common symptoms are related to airway obstruction, dyspnea being the most common, and become more evident when the tracheal lumen is narrowed by more than 75%(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.,99 Dines DE, Lillie JC, Henderson LL, et al. Solitary plasmacytoma of the trachea. Am Rev Respir Dis. 1965;92:949-51.). Other symptoms include cough, dysphonia, hoarseness, hemoptysis, stridor, dysphagia, nasal obstruction, epistaxis, rhinorrhea, ear pain, weight loss, and cyanosis(66 Ferretti GR, Bithigoffer C, Righini CA, et al. Imaging of tumors of the trachea and central bronchi. Radiol Clin North Am. 2009;47:227-41.).

Extramedullary plasmacytoma of the trachea is a rare plasma cell malignancy (accounting for only 4% of plasma cell tumors), having been described in soft tissues outside the bone marrow, involving the submucosal lymphoid tissue, and occurring at different locations, especially in the upper airways, most often in the paranasal sinuses or nose(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.,88 Reyhan M, Tercan F, Ergin M, et al. Sonographic diagnosis of a tracheal extramedullary plasmacytoma. J Ultrasound Med. 2005;24:1031-4.,1010 Sukumaran R, Nair RA, Jacob PM, et al. Extramedullary plasmacytoma of the trachea. Head Neck Pathol. 2014;8:220-4.). Involvement of the larynx, hypopharynx, cervical glands, esophagus, cervical lymph nodes, middle ear, and mastoid is rare(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.), and tracheal involvement is even rarer(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.,1111 Rai SP, Kumar R, Bharadwaj R, et al. Solitary tracheal plasmacytoma. Indian J Chest Dis Allied Sci. 2003;45:269-72.

12 Kober SJ. Solitary plasmacytoma of the carina. Thorax. 1979;34:567-8.
-1313 Kairalla RA, Carvalho CRR, Parada AA, et al. Solitary plasmacytoma of the trachea treated by loop resection and laser therapy. Thorax. 1988; 43:1011-2.), occurring in only 3% of all extramedullary plasmacytomas(99 Dines DE, Lillie JC, Henderson LL, et al. Solitary plasmacytoma of the trachea. Am Rev Respir Dis. 1965;92:949-51.). As of 2005, only 15 cases of solitary extramedullary plasmacytoma of the trachea had been reported in the medical literature(88 Reyhan M, Tercan F, Ergin M, et al. Sonographic diagnosis of a tracheal extramedullary plasmacytoma. J Ultrasound Med. 2005;24:1031-4.). It primarily affects men between 50 and 60 years of age, with a male/female ratio ranging from 3:1 to 5:1(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.,88 Reyhan M, Tercan F, Ergin M, et al. Sonographic diagnosis of a tracheal extramedullary plasmacytoma. J Ultrasound Med. 2005;24:1031-4.). Progression to multiple myeloma is considerably less frequent than is solitary plasmacytoma of the bone(88 Reyhan M, Tercan F, Ergin M, et al. Sonographic diagnosis of a tracheal extramedullary plasmacytoma. J Ultrasound Med. 2005;24:1031-4.).

In ultrasound of the neck, tracheal lesions, especially those located anteriorly, can be visualized clearly(1010 Sukumaran R, Nair RA, Jacob PM, et al. Extramedullary plasmacytoma of the trachea. Head Neck Pathol. 2014;8:220-4.). A CT scan allows the lumen, airway wall, and mediastinal structures to be evaluated. Multiplanar reconstructions are useful for assessing the type, degree, and longitudinal extent of the airway narrowing as well as the location of the tumor and its distance from the cricoid cartilage and carina(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.,77 Olmedo G, Rosenberg M, Fonseca R. Primary tumors of the trachea. Clinicopathologic features and surgical results. Chest. 1982;81:701-6.). Bronchoscopy correlates well with CT and can be used in order to resect the lesion(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.,77 Olmedo G, Rosenberg M, Fonseca R. Primary tumors of the trachea. Clinicopathologic features and surgical results. Chest. 1982;81:701-6.). The diagnosis is made through histological and immunohistochemical studies(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.,1414 Dulmet E, Verley JM, Levasseur P, et al. Solitary plasmacytoma of the trachea. Apropos of a case. Ann Pathol. 1990;10:275-7.). There was one reported case in which the tracheal plasmacytoma was identified as an incidental finding on positron emission tomography/CT(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.). The treatment can be surgical resection alone, radiotherapy alone, requiring annual monitoring, or a combination of the two(55 Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.,1313 Kairalla RA, Carvalho CRR, Parada AA, et al. Solitary plasmacytoma of the trachea treated by loop resection and laser therapy. Thorax. 1988; 43:1011-2.,1414 Dulmet E, Verley JM, Levasseur P, et al. Solitary plasmacytoma of the trachea. Apropos of a case. Ann Pathol. 1990;10:275-7.). There is local recurrence in approximately 30% of cases and metastasis in 15-40%(1313 Kairalla RA, Carvalho CRR, Parada AA, et al. Solitary plasmacytoma of the trachea treated by loop resection and laser therapy. Thorax. 1988; 43:1011-2.).

REFERENCES

  • 1
    Barbosa AGJ, Penha D, Zanetti G, et al. Foreign body in the bronchus of a child: the importance of making the correct diagnosis. Radiol Bras. 2016;49:340-2.
  • 2
    Ribeiro GMR, Natal MRC, Silva EF, et al. Tracheobronchopathia osteochondroplastica: computed tomography, bronchoscopy and histopathological findings. Radiol Bras. 2016;49:56-7.
  • 3
    Barbosa BC, Amorim VB, Ribeiro LFM, et al. Tuberculosis: tracheal involvement. Radiol Bras. 2016;49:410-1.
  • 4
    Franco RM, Guimaraes MD, Moreira BL, et al. Enhancing survival with early surgical resection of endobronchial metastasis in a follow-up of ovarian carcinoma. Radiol Bras. 2015;48:130.
  • 5
    Garelli M, Righini C, Faure C, et al. Imaging of a case of extramedullary solitary plasmacytoma of the trachea. Case Rep Radiol. 2011;2011: 687203.
  • 6
    Ferretti GR, Bithigoffer C, Righini CA, et al. Imaging of tumors of the trachea and central bronchi. Radiol Clin North Am. 2009;47:227-41.
  • 7
    Olmedo G, Rosenberg M, Fonseca R. Primary tumors of the trachea. Clinicopathologic features and surgical results. Chest. 1982;81:701-6.
  • 8
    Reyhan M, Tercan F, Ergin M, et al. Sonographic diagnosis of a tracheal extramedullary plasmacytoma. J Ultrasound Med. 2005;24:1031-4.
  • 9
    Dines DE, Lillie JC, Henderson LL, et al. Solitary plasmacytoma of the trachea. Am Rev Respir Dis. 1965;92:949-51.
  • 10
    Sukumaran R, Nair RA, Jacob PM, et al. Extramedullary plasmacytoma of the trachea. Head Neck Pathol. 2014;8:220-4.
  • 11
    Rai SP, Kumar R, Bharadwaj R, et al. Solitary tracheal plasmacytoma. Indian J Chest Dis Allied Sci. 2003;45:269-72.
  • 12
    Kober SJ. Solitary plasmacytoma of the carina. Thorax. 1979;34:567-8.
  • 13
    Kairalla RA, Carvalho CRR, Parada AA, et al. Solitary plasmacytoma of the trachea treated by loop resection and laser therapy. Thorax. 1988; 43:1011-2.
  • 14
    Dulmet E, Verley JM, Levasseur P, et al. Solitary plasmacytoma of the trachea. Apropos of a case. Ann Pathol. 1990;10:275-7.

Publication Dates

  • Publication in this collection
    Jan-Feb 2017
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