AM ER IC AN JOURNAL OF OT OLARYNGOLOGY – H E A D A N D NE CK M E D IC IN E A ND S U RGE RY 3 6 (2 0 1 5) 2 87– 2 8 9
Available online at www.sciencedirect.com
ScienceDirect www.elsevier.com/locate/amjoto
Primary fibrosarcoma of the trachea presenting with acute airway loss Conner Massey, BS a,⁎, Nora Laver, MD b , Harprit Bedi, MD c , Richard O. Wein, MD, FACS d a
Tufts University School of Medicine, 145 Harrison Ave, Boston, MA Department of Ophthalmology, Tufts Medical Center c Department of Radiology, Tufts Medical Center d Department of Otolaryngology – HNS, Tufts Medical Center b
ARTI CLE I NFO
A BS TRACT
Article history:
Primary sarcomas of the trachea are rare occurrences that present with nonspecific
Received 3 October 2014
symptoms, making timely diagnosis difficult. We report a case of primary fibrosarcoma of the trachea that presented with acute airway loss secondary to tracheal discontinuity due to tumor destruction. This unusual clinical presentation highlights the difficulties posed in the diagnosis and management of tracheal sarcomas. A discussion of the relevant literature is presented. © 2015 Elsevier Inc. All rights reserved.
1.
Introduction
The vast majority of soft tissue sarcomas originate in the limbs and trunk with only 11% presenting as primary tumors of the head and neck [1]. Sarcomas of the trachea are quite rare and encompass only 3.6% of all primary tracheal tumors. The diagnosis of fibrosarcoma involving the trachea is uncommon and is often a diagnosis of exclusion. We present here a case of primary fibrosarcoma of the trachea that resulted in acute airway compromise.
2.
Case report
The Otolaryngology service was consulted to see a 68 year-old female that was admitted with complaints of persistent shortness of breath and intermittent hoarseness. The patient had a complex medical history including tracheal stenosis after tracheostomy for which she had undergone tracheal dilation 3 months prior to her admission.
The patient’s airway was initially evaluated with flexible fiberoptic laryngoscopy and demonstrated no laryngeal abnormalities with normal true vocal mobility. Over the subsequent 48 h, the patient experienced worsening shortness of breath and was transferred to ICU level care. CT imaging of the neck and chest was scheduled with a plan for intubation prior to transport. However, at the time of attempted transoral intubation, the endotracheal tube could not be passed below the level of the glottis and ENT was emergently called to perform bedside tracheostomy. After the initial dissection in the lower midline neck, extensive soft tissue necrosis with destruction of the cricoid cartilage and tracheal separation was noted. Following removal of necrotic debris, an endotracheal tube was introduced into the mediastinal aspect of the trachea. The patient remained in stable condition following the procedure despite intermittent air leaks that resulted from the compromised stability of the distal trachea. Subsequent imaging demonstrated marked irregularity and discontinuity of the anterior tracheal wall, as well as non-specific linear hyperdensity
⁎ Corresponding author at: Tufts University School of Medicine, 145 Harrison Ave, Boston, MA 02111. Tel.: +1 4144912826. E-mail address: [email protected] (C. Massey). http://dx.doi.org/10.1016/j.amjoto.2014.10.023 0196-0709/© 2015 Elsevier Inc. All rights reserved.
288
AM ER IC AN JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D NE CK M E D ICI N E AN D S U RGE RY 3 6 (2 0 1 5) 2 87 – 2 8 9
along the anterior tracheal margin, but no obvious mass lesions (Figs. 1, 2). The patient was taken for subsequent tracheostomy revision as well as neck exploration. Microlaryngoscopy demonstrated near-total destruction of the cricoid and superior segments of the trachea. The neck was then explored and numerous tissue biopsies were performed. The anterior trachea was mobilized from the mediastinum and sewn to an advancement skin flap from the chest. Biopsies demonstrated a tumor composed of a population of spindle cells with pale cytoplasms and ovoid to elongated, tapered nuclei focally arranged in fascicular patterns with collagen deposition and areas of necrosis. The tumor cells showed immunoreactivity with vimentin only. SMA, MSA, desmin, CD34, S-100, pankeratin, CD117 (c-kit), synaptophysin TTF-1, CD99, CD45, CK5/6, CAM 5.2, EMA, p63 and ALK stains were negative, ruling out a spindle cell carcinoma, spindle cell malignant melanoma, leiomyosarcoma, monophasic synovial sarcoma, malignant peripheral nerve sheath tumor, liposarcoma, and metastatic tumors. The histologic features, coupled with the negative immunohistochemical profile are consistent with a diagnosis of poorly differentiated fibrosarcoma. Anaplastic thyroid carcinoma was strongly considered in the differential, but was deemed unlikely given the tumor cytology, low mitotic (less than 2 mitoses per 10 HPF) and ki-67 indices and absence of p53 staining (Fig. 3). Postoperatively, the patient remained on ventilatory support and suffered from persistent pulmonary secretions. Possible therapeutic procedures, including total laryngectomy with manubrium resection for stoma placement, were discussed but eventually precluded from consideration given the patient’s other co-morbidities and overall poor prognosis. The patient remained on respiratory support for two more weeks, at which point the patient’s family elected to withdraw care.
Fig. 1 – Axial CT of neck soft tissue demonstrates a tracheostomy tube in place with marked irregularity and discontinuity of the anterior tracheal wall. Air is noted in the midline projecting into the thyroid bed.
Fig. 2 – Axial CT of neck soft tissue at the level of the thyroid demonstrates a non-specific linear hyperdensity along the anterior tracheal margin, possibly originating from the trachea or the thyroid. There is no obvious mass associated with the trachea. There is also a large calcification in the left lobe of thyroid. Mild rightward deviation of the trachea is noted.
3.
Discussion
Neoplastic tracheal lesions can be difficult to diagnose as they can often present with nonspecific symptoms. One retrospective case series found that among malignant primaries of the trachea, the most common presenting symptoms were hemoptysis (54%) and cough (50%). Dyspnea and hoarseness, the presenting complaints of our patient, were much less common at 38% and 7%, respectively. The nonspecific symptomatology of these tumors and the lack of identifiable
Fig. 3 – Hematoxylin and eosin stain demonstrating a spindle cell population with focal fibrous tissue deposition and fascicular growth pattern, cellular pleomorphism with increased mitotic activity, and extensive hemorrhage and necrosis.
AM ER IC AN JOURNAL OF OT OLARYNGOLOGY – H E A D A N D NE CK M E D IC IN E A ND S U RGE RY 3 6 (2 0 1 5) 2 87– 2 8 9
risk factors delay the time to diagnosis. For patients with tracheal sarcomas, the mean duration of time from onset of symptoms to diagnosis was 7 months. The study further showed that while the 5-year survival rate for resectable tracheal sarcomas was 78%, unresectable tumors were uniformly fatal [2]. A review of the literature demonstrates several cases of tracheal sarcomas initially presenting with acute airway loss. Rana et al. report a case of a 25 year-old male with a threeweek history of hemoptysis and dyspnea who presented with acute respiratory distress due to left lung collapse [3]. The patient was subsequently found to have a pedunculated spindle cell sarcoma originating from the distal trachea that had prolapsed into the left main bronchus. Emergent surgery was undertaken, permitting complete excision of the tumor. Elmi et al. reported on a patient that presented acute respiratory distress with CT findings of a large exophytic mass obstructing the tracheal lumen, which was later diagnosed as an extraskeletal Ewing’s sarcoma [4]. Bronchoscopic debridement of the mass failed to re-establish the airway, which was eventually secured with a silicone “Y”-tracheobronchial stent. This allowed for a course of neo-adjuvant chemotherapy and radiation prior to complete surgical excision. The diagnosis of fibrosarcoma is an uncommon diagnosis in the head and neck region. It typically presents in the 4th to 6th decade of life in the extremities or trunk and is more common in males. History of prior radiation therapy has been reported as an etiology for the development of fibrosarcoma, however in most cases no clear etiology exists. Surgery remains the mainstay of treatment with adjuvant radiation and chemotherapy utilized in selected presentations [5]. Our case is unique in that it describes an instance of acute airway loss due not to mass effect, but rather due to near-total obliteration and tracheal separation that became evident at the time of intubation. Patients presenting with what initially
289
appears to be acquired tracheal stenosis, either secondary to prior tracheostomy or trauma, should be considered candidates for biopsy at the time of tracheal dilation. Such an approach would rule out the possibility of neoplastic disease, a rare, yet serious, etiology for tracheal stenosis.
4.
Conclusion
Primary sarcomas of trachea are uncommon entities that can present with nonspecific symptoms, making accurate and timely diagnosis difficult. In instances of acute airway compromise due to these tumors, surgical management is particularly challenging, especially when pre-operative imaging is not possible. Our case further highlights the limited therapeutic options and dismal outcome when locally advanced tumors result in destruction rather than obstruction of the airway. For patients with acquired tracheal stenosis, biopsy at the time of tracheal dilation should be considered to rule out neoplastic pathology.
REFERENCES
[1] Ducimetière F, Lurkin A, Ranchère-Vince D, et al. Incidence of sarcoma histotypes and molecular subtypes in a prospective epidemiological study with central pathology review and molecular testing. PLoS One 2011;6:e20294. [2] Gaissert HA, Grillo HC, Shadmehr MB, et al. Uncommon primary tracheal tumors. Ann Thorac Surg 2006;82:268–72. [3] Rana SS, Balamurali S, Dronamraju VA, et al. Unusual malignant tracheal tumour presenting with acute airway obstruction. Indian J Chest Dis Allied Sci 2007;49:49–51. [4] Elmi M, Ko MA, Gupta A, et al. Primary tracheal Ewing’s sarcoma. Ann Thorac Surg 2010;90:1349–52. [5] Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol 2003;15:239–52.
Available online at www.sciencedirect.com
ScienceDirect www.elsevier.com/locate/amjoto
Primary fibrosarcoma of the trachea presenting with acute airway loss Conner Massey, BS a,⁎, Nora Laver, MD b , Harprit Bedi, MD c , Richard O. Wein, MD, FACS d a
Tufts University School of Medicine, 145 Harrison Ave, Boston, MA Department of Ophthalmology, Tufts Medical Center c Department of Radiology, Tufts Medical Center d Department of Otolaryngology – HNS, Tufts Medical Center b
ARTI CLE I NFO
A BS TRACT
Article history:
Primary sarcomas of the trachea are rare occurrences that present with nonspecific
Received 3 October 2014
symptoms, making timely diagnosis difficult. We report a case of primary fibrosarcoma of the trachea that presented with acute airway loss secondary to tracheal discontinuity due to tumor destruction. This unusual clinical presentation highlights the difficulties posed in the diagnosis and management of tracheal sarcomas. A discussion of the relevant literature is presented. © 2015 Elsevier Inc. All rights reserved.
1.
Introduction
The vast majority of soft tissue sarcomas originate in the limbs and trunk with only 11% presenting as primary tumors of the head and neck [1]. Sarcomas of the trachea are quite rare and encompass only 3.6% of all primary tracheal tumors. The diagnosis of fibrosarcoma involving the trachea is uncommon and is often a diagnosis of exclusion. We present here a case of primary fibrosarcoma of the trachea that resulted in acute airway compromise.
2.
Case report
The Otolaryngology service was consulted to see a 68 year-old female that was admitted with complaints of persistent shortness of breath and intermittent hoarseness. The patient had a complex medical history including tracheal stenosis after tracheostomy for which she had undergone tracheal dilation 3 months prior to her admission.
The patient’s airway was initially evaluated with flexible fiberoptic laryngoscopy and demonstrated no laryngeal abnormalities with normal true vocal mobility. Over the subsequent 48 h, the patient experienced worsening shortness of breath and was transferred to ICU level care. CT imaging of the neck and chest was scheduled with a plan for intubation prior to transport. However, at the time of attempted transoral intubation, the endotracheal tube could not be passed below the level of the glottis and ENT was emergently called to perform bedside tracheostomy. After the initial dissection in the lower midline neck, extensive soft tissue necrosis with destruction of the cricoid cartilage and tracheal separation was noted. Following removal of necrotic debris, an endotracheal tube was introduced into the mediastinal aspect of the trachea. The patient remained in stable condition following the procedure despite intermittent air leaks that resulted from the compromised stability of the distal trachea. Subsequent imaging demonstrated marked irregularity and discontinuity of the anterior tracheal wall, as well as non-specific linear hyperdensity
⁎ Corresponding author at: Tufts University School of Medicine, 145 Harrison Ave, Boston, MA 02111. Tel.: +1 4144912826. E-mail address: [email protected] (C. Massey). http://dx.doi.org/10.1016/j.amjoto.2014.10.023 0196-0709/© 2015 Elsevier Inc. All rights reserved.
288
AM ER IC AN JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D NE CK M E D ICI N E AN D S U RGE RY 3 6 (2 0 1 5) 2 87 – 2 8 9
along the anterior tracheal margin, but no obvious mass lesions (Figs. 1, 2). The patient was taken for subsequent tracheostomy revision as well as neck exploration. Microlaryngoscopy demonstrated near-total destruction of the cricoid and superior segments of the trachea. The neck was then explored and numerous tissue biopsies were performed. The anterior trachea was mobilized from the mediastinum and sewn to an advancement skin flap from the chest. Biopsies demonstrated a tumor composed of a population of spindle cells with pale cytoplasms and ovoid to elongated, tapered nuclei focally arranged in fascicular patterns with collagen deposition and areas of necrosis. The tumor cells showed immunoreactivity with vimentin only. SMA, MSA, desmin, CD34, S-100, pankeratin, CD117 (c-kit), synaptophysin TTF-1, CD99, CD45, CK5/6, CAM 5.2, EMA, p63 and ALK stains were negative, ruling out a spindle cell carcinoma, spindle cell malignant melanoma, leiomyosarcoma, monophasic synovial sarcoma, malignant peripheral nerve sheath tumor, liposarcoma, and metastatic tumors. The histologic features, coupled with the negative immunohistochemical profile are consistent with a diagnosis of poorly differentiated fibrosarcoma. Anaplastic thyroid carcinoma was strongly considered in the differential, but was deemed unlikely given the tumor cytology, low mitotic (less than 2 mitoses per 10 HPF) and ki-67 indices and absence of p53 staining (Fig. 3). Postoperatively, the patient remained on ventilatory support and suffered from persistent pulmonary secretions. Possible therapeutic procedures, including total laryngectomy with manubrium resection for stoma placement, were discussed but eventually precluded from consideration given the patient’s other co-morbidities and overall poor prognosis. The patient remained on respiratory support for two more weeks, at which point the patient’s family elected to withdraw care.
Fig. 1 – Axial CT of neck soft tissue demonstrates a tracheostomy tube in place with marked irregularity and discontinuity of the anterior tracheal wall. Air is noted in the midline projecting into the thyroid bed.
Fig. 2 – Axial CT of neck soft tissue at the level of the thyroid demonstrates a non-specific linear hyperdensity along the anterior tracheal margin, possibly originating from the trachea or the thyroid. There is no obvious mass associated with the trachea. There is also a large calcification in the left lobe of thyroid. Mild rightward deviation of the trachea is noted.
3.
Discussion
Neoplastic tracheal lesions can be difficult to diagnose as they can often present with nonspecific symptoms. One retrospective case series found that among malignant primaries of the trachea, the most common presenting symptoms were hemoptysis (54%) and cough (50%). Dyspnea and hoarseness, the presenting complaints of our patient, were much less common at 38% and 7%, respectively. The nonspecific symptomatology of these tumors and the lack of identifiable
Fig. 3 – Hematoxylin and eosin stain demonstrating a spindle cell population with focal fibrous tissue deposition and fascicular growth pattern, cellular pleomorphism with increased mitotic activity, and extensive hemorrhage and necrosis.
AM ER IC AN JOURNAL OF OT OLARYNGOLOGY – H E A D A N D NE CK M E D IC IN E A ND S U RGE RY 3 6 (2 0 1 5) 2 87– 2 8 9
risk factors delay the time to diagnosis. For patients with tracheal sarcomas, the mean duration of time from onset of symptoms to diagnosis was 7 months. The study further showed that while the 5-year survival rate for resectable tracheal sarcomas was 78%, unresectable tumors were uniformly fatal [2]. A review of the literature demonstrates several cases of tracheal sarcomas initially presenting with acute airway loss. Rana et al. report a case of a 25 year-old male with a threeweek history of hemoptysis and dyspnea who presented with acute respiratory distress due to left lung collapse [3]. The patient was subsequently found to have a pedunculated spindle cell sarcoma originating from the distal trachea that had prolapsed into the left main bronchus. Emergent surgery was undertaken, permitting complete excision of the tumor. Elmi et al. reported on a patient that presented acute respiratory distress with CT findings of a large exophytic mass obstructing the tracheal lumen, which was later diagnosed as an extraskeletal Ewing’s sarcoma [4]. Bronchoscopic debridement of the mass failed to re-establish the airway, which was eventually secured with a silicone “Y”-tracheobronchial stent. This allowed for a course of neo-adjuvant chemotherapy and radiation prior to complete surgical excision. The diagnosis of fibrosarcoma is an uncommon diagnosis in the head and neck region. It typically presents in the 4th to 6th decade of life in the extremities or trunk and is more common in males. History of prior radiation therapy has been reported as an etiology for the development of fibrosarcoma, however in most cases no clear etiology exists. Surgery remains the mainstay of treatment with adjuvant radiation and chemotherapy utilized in selected presentations [5]. Our case is unique in that it describes an instance of acute airway loss due not to mass effect, but rather due to near-total obliteration and tracheal separation that became evident at the time of intubation. Patients presenting with what initially
289
appears to be acquired tracheal stenosis, either secondary to prior tracheostomy or trauma, should be considered candidates for biopsy at the time of tracheal dilation. Such an approach would rule out the possibility of neoplastic disease, a rare, yet serious, etiology for tracheal stenosis.
4.
Conclusion
Primary sarcomas of trachea are uncommon entities that can present with nonspecific symptoms, making accurate and timely diagnosis difficult. In instances of acute airway compromise due to these tumors, surgical management is particularly challenging, especially when pre-operative imaging is not possible. Our case further highlights the limited therapeutic options and dismal outcome when locally advanced tumors result in destruction rather than obstruction of the airway. For patients with acquired tracheal stenosis, biopsy at the time of tracheal dilation should be considered to rule out neoplastic pathology.
REFERENCES
[1] Ducimetière F, Lurkin A, Ranchère-Vince D, et al. Incidence of sarcoma histotypes and molecular subtypes in a prospective epidemiological study with central pathology review and molecular testing. PLoS One 2011;6:e20294. [2] Gaissert HA, Grillo HC, Shadmehr MB, et al. Uncommon primary tracheal tumors. Ann Thorac Surg 2006;82:268–72. [3] Rana SS, Balamurali S, Dronamraju VA, et al. Unusual malignant tracheal tumour presenting with acute airway obstruction. Indian J Chest Dis Allied Sci 2007;49:49–51. [4] Elmi M, Ko MA, Gupta A, et al. Primary tracheal Ewing’s sarcoma. Ann Thorac Surg 2010;90:1349–52. [5] Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol 2003;15:239–52.
