DOI:10.2214/AJR.05.1116
AJR 2006; 187:W231-W232
© American Roentgen Ray Society
Tracheal Paraganglioma: A Rare Vascular Neoplasm
Mercy George,
Anoop Padoor Ayyappan,
Rekha Cherian and
Mary Kurien
Christian Medical College and Hospital Tamil Nadu, India
Paragangliomas are neoplasms arising from extraadrenal chromaffin cells.
Laryngeal and tracheal paragangliomata are rare neoplasms of branchiomeric
origin. They are innervated by the parasympathetic nervous system and only
rarely secrete catecholamines. This case of tracheal paraganglioma illustrates
certain preoperative diagnostic clues provided by imaging that significantly
contributed to better patient management.
A 22-year-old man presented to the department of otorhinolaryngology with
respiratory difficulty and intermittent hemoptysis of 11-months' duration.
Clinical assessment revealed a biphasic stridor. Fiberoptic
nasopharyngo-laryngoscopic examination showed a pedicled, polypoid lesion with
a bluish hue attached to the posterior wall of the trachea. The lesion was
seen moving up and down during respiration. A vascular neoplasmlike hemangioma
was considered. Axial CT showed an intensely enhancing 2-cm polypoid tumor
about 1 inch (2.54 cm) below the level of the vocal cords in the trachea
(Figs. 3A and
3B). The tumor's rich
vascularity led us to consider an unusual neoplasm of the trachea such as a
paraganglioma, hemangioma, carcinoid, or hemangiopericytoma, and a biopsy was
deferred. The estimation of urinary vanillylmandelic acid level was normal. At
surgery, a tracheostomy was performed for securing the airway, and the tumor
was dissected off the posterior tracheal wall through a tracheofissure. The
lesion was limited to the trachea and with no involvement of the adjacent
thyroid gland. The base of the lesion was cauterized with diathermy. Histology
of the lesion revealed tumor cells with abundant eosinophilic cytoplasm
arranged in clusters (zellballen pattern) interspersed with fibrovascular
stroma in keeping with a paraganglioma (Figs.
3A and
3B). Convalescence was
uneventful.
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Fig. 3B —22-year-old man with tracheal paraganglioma. Photomicrograph
shows tumor cells with abundant eosinophilic cytoplasm, round nuclei, and
finely granular chromatin with focal anisokaryosis. Note characteristic
nesting pattern of tumor cells (zellballen pattern). (H and E, x400)
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Paragangliomas in the trachea are exceedingly rare and only a few cases
have been reported in the medical literature. They usually pressent between
the fourth to sixth decades of life and are three to four times more common in
women [1]. Patients often
present with intermittent hemoptysis and symptoms relating to airway narrowing
[2].
Paragangliomas are vascular tumors. In most of the documented cases,
including our case, the tumor revealed a predisposition to originate from the
posterior wall of the trachea
[2]. Its biologic behavior is
almost always benign, with less than 2% showing malignant features
[3].
In cases of tracheal paragangliomas, surgical excision is the recommended
treatment [2]. Preoperative
embolization can be used to devascularize the lesion before removal
[4]. Airway management during
surgery poses a problem for patients with vascular neoplasms of the trachea.
There exists a risk of injuring the lesion during intubation. This can result
in hemorrhage or cause a fragment of the lesion to be inhaled by the patient
[2]. Either situation could
lead to severe compromise of the unprotected distal airway. Bearing in mind
the above-mentioned associated risks, we performed a tracheostomy to secure
the airway in our patient.
In conclusion, a preoperative suspicion of a highly vascular neoplasm in
the trachea helped us to avoid performing a biopsy. It also facilitated
meticulous surgical planning, thereby enabling us to adequately manage the
airway resulting in a successful excision of the paraganglioma without any
complication.
References
- Ferlito A, Rosai J. Terminology and classification of
neuroendocrine neoplasms of the larynx. ORL J Otorhinolaryngol
Relat Spec 1991; 53:185
-187[Medline]
- Jones TM, Alderson D, Sheard JD, Swift AC. Tracheal paraganglioma:
a diagnostic dilemma culminating in a complex airway management problem.
J Laryngol Otol 2001;115
: 747-749[CrossRef][Medline]
- Ferlito A, Barnes L, Rinaldo A, Gnepp DR, Milroy CM. A review of
neuroendocrine neoplasms of the larynx: update on diagnosis and treatment.
J Laryngol Otol 1998;112
: 827-834[Medline]
- Michaelson PG, Fowler CB, Brennan J. Tracheal paraganglioma
presenting with acute airway obstruction. Otolaryngol Head Neck
Surg 2005; 132:661
-662[CrossRef][Medline]
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