Laryngeal Myxoma Thomas Sena, MD; Mary Susan Brady, MD; Andrew G. Huvos, MD; Ronald H. Spiro, MD
\s=b\ Myxoma is a rare mesenchymal tumor that is especially uncommon in the head and neck. We report a case of a myxoma of the larynx in a 70-year-old man, which, to our knowledge, is the second case reported in the English-language literature. Although benign, myxomas have a propensity for local recurrence due to their tendency to infiltrate surrounding tissues. They commonly appear encapsulated, but simple enucleation is not adequate treatment; excision should include a margin of normal tissue around the tumor. (Arch Otolaryngol Head Neck Surg. 1991;
117:430-432)
Myxoma Virchow,
was
in
first described
1871,
as
a
by
tumor
whose histologie appearance resem¬ bled the mucinous substance of the umbilical cord.1 In 1948, Stout2 re¬ ported a series of patients with myxo¬ ma and outlined diagnostic criteria. He defined myxomas as neoplasms of mes¬ enchymal origin that did not metastasize and described them as "tumors Accepted for publication December 3, 1990. From the Head and Neck Service, Department of Surgery (Drs Sena, Brady, and Spiro), and the Department of Pathology (Dr Huvos), Memorial Sloan-Kettering Cancer Center, New York, NY. Dr Sena is a surgical resident, New York Medical College, Valhalla, NY. Reprint requests to Head and Neck Service, Memorial Sloan-Kettering Cancer Center, 1275 York Ave, New York, NY 10021 (Dr Spiro).
composed of stellate cells set in a loose mucoid stroma."2 Today, myxomas are considered benign tumors of mesenchymal origin that occur in somatic soft tissue and bone, although myxomas of bone are rare outside of the facial skeleton.34 Myxomas of soft tissue occur in skele¬ tal muscle, subcutaneous tissue, fasciai planes, and neurovascular sheaths and tend to arise in the extremities.3·' When a myxoma occurs in the head and neck, the most common locations are the man¬ dible and maxilla, but it can also arise in the soft tissue of the face and neck, the nasal cavity, parotid gland, and oral mucosa.12 Myxoma involving the larynx is distinctly unusual; there is, to our knowledge, one prior report in the En¬ glish-language literature concerning this entity." We describe a case of a laryn¬ geal myxoma that developed in a 70year-old man and review the literature on this subject. REPORT OF A CASE A 70-year-old man presented to Memorial Sloan-Kettering Cancer Center, New York, NY, complaining of hoarseness of 3 months' duration. He had a 10-pack-year history of smoking and moderate use of alcohol. He denied symptoms of hemoptysis, weight
loss, or stridor. On physical examination, his neck was supple, without lymphadenopathy or thyromegaly. There were no lesions in the mouth
Downloaded From: http://archotol.jamanetwork.com/ by a New York University User on 06/22/2015
oropharynx. Indirect laryngoscopy re¬ vealed an epiglottis shifted to the right, with obliteration of the left aryepiglottic fold by a large, well-circumscribed, mobile mass. The left vocal cord was difficult to visualize due to obstruction of the vestibule. A computed tomographic scan of the neck demonstrated a tumor of the left supraglottic larynx consistent with a fluid-filled laryngoeele (Fig 1). The patient was taken to the operating room for excision of the mass. A nasotracheal tube was placed endoscopically, and direct laryngoscopy was performed. A large tumor was noted in the left supraglottic area, oc¬ cluding the laryngeal vestibule. The overly¬ ing mucosa appeared normal. Needle aspira¬ tion of the mass yielded only a small amount of mucoid material, which was submitted for cytologie analysis. A transverse incision was made in the skin, between the hyoid bone and the thyroid cartilage, and a 6-cm cystic tumor was then easily palpable beneath the strap muscles. The mass was dissected free of the surrounding tissues and the epiglottis to the level of the thyroid cartilage. The excised tumor was lobular and had the consistency of a mucocele. A frozen-section specimen was consistent with a lipoma. Gross examination revealed a 6.5 5.0 2.5-cm mass, with a thin, fibrous capsule and a mottled, nodular surface (Fig 2). On sec¬ tioning, the tumor was composed of grayishwhite, gelatinous material characteristic of myxoma. The diagnosis was confirmed on paraffin section (Fig 3). Postoperatively, the patient did well, with gradual lessening of his hoarseness. He re¬ mains well 12 months after his operation. or
Fig 1 Computed tomographic scan of myxoma of the supraglottic larynx. Left. Axial section at the level of the hyoid bone. Right. Lower axial section just above level of vocal cords. —
.
fibroxanthomas, liposarcomas, fibrous
dysplasia, or nodular fasciitis. Histologically, myxomas contain in¬ conspicuous stellate cells with bland '"''
nuclei, abundant mucoid material that stains
positive for mucopolysaccharides, and a rich network of delicate
reticular fibers."" Characteristics of a preclude the diagnosis of include the presence of myomyxoma blasts, lipoblasts, or chondroblasts.2 A myxoid lesion with evidence of malig¬ nancy is usually a sarcoma with areas of myxoid change, often a liposartumor that
Gross appearance of myxoma following
Fig 2.
resection. —
Fig 3. —Histologie section demonstrating both collagenous and myxomatous elements within the tumor (hematoxylin-eosin, 250).
coma."
histologie differential diagnosis laryngeal myxoma includes the myxoid type of vocal cord polyp, with which it is commonly mistaken. Un¬ like myxomas, vocal cord polyps dis¬ play prominent blood vessels and fre¬ quently feature subepithelial basement membrane hyalinization. Myxoid vocal cord polyps are always covered by stratified squamous epithelium, in con¬ The
of COMMENT The most common clinical presenta¬ tion of a myxoma is that of a slowly growing, painless mass.' Myxomas of the head and neck occur with equal frequency in men and women and tend to occur in the third and fourth de¬ cades of life, although individuals of all ages can be affected.L2J Babbitt and Pfeiffer" reported a myxoma of the palate in a 14-year-old female patient; our patient with a laryngeal myxoma was a
70-year-old
man.
The origin of myxomas is unknown, but antecedent infection or trauma has been proposed as initiating events.' Myxomas may be tumors of primitive
mesenchyme,
a
concept supported by
the gross and histologie similarities between the two tissues. The tumor contains delicate, interlacing reticular fibers and abundant mucopolysaccharide characteristic of primitive mesen¬ chyme.1' Myxomas of the mandible of¬ ten occur around erupting teeth and contain odontogenic epithelium; they may also arise from odontogenic, pri¬ mordial mesenchyme." Grossly, myxomas are gray, white, or opalescent, gelatinous tumors that may appear encapsulated due to com¬ pression of surrounding tissue.3 They are typically rubbery in texture, but can range from soft to firm in consis¬ tency, and often resemble fibromas,
a
trast to myxomas, which may or may not be lined with such epithelium. In
addition, myxoid vocal cord polyps al¬ ways
occur on
the true vocal cord.
Myxomas of the head and neck usu¬ ally arise in the mandible or maxilla, but they can also occur in the parotid gland, orbit, mucosa of the oral cavity and oropharynx, or soft tissues of the face and neck.121" The only other case of a laryngeal myxoma reported in the
Downloaded From: http://archotol.jamanetwork.com/ by a New York University User on 06/22/2015
English-language literature was that by Chen and Ballecer," in 1986. It occurred in a 37-year-old man who complained of dysphagia and dysphonia secondary to a pedunculated myxo¬ ma of the lingual epiglottis. The treatment of a myxoma depends on
its location and size. Due to their
encapsulated appearance, myxomas are often simply enucleated. The pseu¬ docapsule around the tumor is rarely complete, however, and local recur¬ rence may result, usually within the first 3 years.3 Myxomas of bone may have a particularly high rate of recur¬
Zimmerman and Dahlin7 re¬ ported a series of 20 patients with myxomas of the jaw: four of those patients had recurrent tumors on pre¬ sentation, and in four patients tumors recurred following the first or second excision. Ten patients underwent re¬ section of myxomas of the mandible or maxilla at Memorial Sloan-Kettering Cancer Center. Nine patients had their tumors excised with a portion of rence.
the
underlying bone,
recurrence
at 10
without tumor years. One local excision
or more
patient underwent a only, and in this patient
tumor
re¬
curred 5 years later.'" Successful resection of a myxoma of the facial bones mandates adequate exposure. Keller12 discusses the resec¬ tion of a myxoma of the maxilla by means of a Lefort I osteotomy. Petti and coworkers13 describe the resection of a mandibular myxoma using a sagit¬ tal ramus osteotomy. Myxomas of soft tissue are probably less likely to recur following resection.3 Ireland et al' reported only two recur¬ rences of tumors in 45 examined pa¬ tients.' Removing the tumor with a margin of normal tissue will minimize the risk of local recurrence; frozensection analysis of questionable mar¬ gins should be used to ensure adequate resection. Multiple myxomas of the head and neck that occur in a young patient should alert the clinician to the possi-
bility of a systemic disorder character¬ ized by cardiac- and soft-tissue myxo¬
(often of the head and neck), facial and labial skin pigmentation, and en¬ docrine overactivity (Cushing's syn¬
mas
drome, acromegaly).
"
Carney
et al15
series of 40 patients with this disorder, which appears to be in¬ herited in an autosomal dominant fash¬ ion. Of note is that 25% of the patients died of disease secondary to emboliza¬ tion from a cardiac myxoma. In summary, myxomas of the head and neck are uncommon lesions, with
reported
a
laryngeal tumors being particularly rare. Although benign, their propensi¬ ty for local recurrence mandates good intraoperative exposure and excision with a margin of normal tissue. When facial and labial pigmentation and/or signs of endocrine overactivity appear, the patient with a head and neck myx¬ oma should be examined for myxoma.
a
cardiac
References 1. Canalis RF, Smith GA, Konrad HR. Myxoof the head and neck. Arch Otolaryngol.
mas
1976;102:300-305. 2. Stout AP. Myxoma tumor of primitive mesenchyme. Ann Surg. 1948;127:706-719. 3. Batsakis JG. Myxomas of soft tissues and the facial skeleton. Ann
Otol Rhinol
Laryngol.
1987;96:618-619. 4. McClure DK, Dahlin DC. Myxoma of bone. Mayo Clin Proc. 1977;52:249-253. 5. Ireland DCR, Soule EH, Ivins JC. Myxoma of somatic soft tissues. Mayo Clin Proc. 1973;48:
401-410. 6. Chen KTK, Ballecer RA. Laryngeal myxoma. Am J Otolaryngol. 1986;7:58-59. 7. Zimmerman DC, Dahlin DC. Myxomatous tu-
of the jaws. Oral Surg Oral Med Oral Pathol. 1958;11:1069-1080. 8. Babbitt JA, Pfeiffer DB. Myxoma of the pal-
mors
ate and
pharynx.
Arch
Otolaryngol. 1937;26:
453-458. 9. Fu YS, Perzin KH. Non-epithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx: a clinico-pathologic study, VII: myxomas. Cancer. 1977;39:195-203. 10. Ghosh BC, Huvos AG, Gerold FP, Miller TR. Myxoma of the jaw bones. Cancer.
1973;31:237-240. 11. Robbins SL, Cotran RS, Kumar V. Clinical aspects of neoplasia. In: Pathologic Basis of Disease. 3rd ed. Philadelphia, Pa: WB Saunders Co; 1984:270.
Downloaded From: http://archotol.jamanetwork.com/ by a New York University User on 06/22/2015
12. Keller EE. Resection of a myxoma of the maxilla via Lefort I osteotomy. J Oral Maxillofac
Surg. 1988;46:609-613.
13. Petti NA, Weber FL, Miller MC. Resection of a mandibular myxoma via a sagittal ramus osteotomy. J Oral Maxillofac Surg. 1987;45:793-795. 14. Cook CA, Lund BA, Carney JA. Mucocutaneous pigmented spots and oral myxomas: the oral manifestations of the complex of myxomas, spotty pigmentation and endocrine overactivity. Oral Surg Oral Med Oral Pathol. 1987;63:175-183. 15. Carney JA, Hruska LS, Beauchamp GD, Gordon H. Dominant inheritance of the complex of myxomas, spotty pigmentation, and endocrine overactivity. Mayo Clin Proc. 1986;61:165-172.
\s=b\ Myxoma is a rare mesenchymal tumor that is especially uncommon in the head and neck. We report a case of a myxoma of the larynx in a 70-year-old man, which, to our knowledge, is the second case reported in the English-language literature. Although benign, myxomas have a propensity for local recurrence due to their tendency to infiltrate surrounding tissues. They commonly appear encapsulated, but simple enucleation is not adequate treatment; excision should include a margin of normal tissue around the tumor. (Arch Otolaryngol Head Neck Surg. 1991;
117:430-432)
Myxoma Virchow,
was
in
first described
1871,
as
a
by
tumor
whose histologie appearance resem¬ bled the mucinous substance of the umbilical cord.1 In 1948, Stout2 re¬ ported a series of patients with myxo¬ ma and outlined diagnostic criteria. He defined myxomas as neoplasms of mes¬ enchymal origin that did not metastasize and described them as "tumors Accepted for publication December 3, 1990. From the Head and Neck Service, Department of Surgery (Drs Sena, Brady, and Spiro), and the Department of Pathology (Dr Huvos), Memorial Sloan-Kettering Cancer Center, New York, NY. Dr Sena is a surgical resident, New York Medical College, Valhalla, NY. Reprint requests to Head and Neck Service, Memorial Sloan-Kettering Cancer Center, 1275 York Ave, New York, NY 10021 (Dr Spiro).
composed of stellate cells set in a loose mucoid stroma."2 Today, myxomas are considered benign tumors of mesenchymal origin that occur in somatic soft tissue and bone, although myxomas of bone are rare outside of the facial skeleton.34 Myxomas of soft tissue occur in skele¬ tal muscle, subcutaneous tissue, fasciai planes, and neurovascular sheaths and tend to arise in the extremities.3·' When a myxoma occurs in the head and neck, the most common locations are the man¬ dible and maxilla, but it can also arise in the soft tissue of the face and neck, the nasal cavity, parotid gland, and oral mucosa.12 Myxoma involving the larynx is distinctly unusual; there is, to our knowledge, one prior report in the En¬ glish-language literature concerning this entity." We describe a case of a laryn¬ geal myxoma that developed in a 70year-old man and review the literature on this subject. REPORT OF A CASE A 70-year-old man presented to Memorial Sloan-Kettering Cancer Center, New York, NY, complaining of hoarseness of 3 months' duration. He had a 10-pack-year history of smoking and moderate use of alcohol. He denied symptoms of hemoptysis, weight
loss, or stridor. On physical examination, his neck was supple, without lymphadenopathy or thyromegaly. There were no lesions in the mouth
Downloaded From: http://archotol.jamanetwork.com/ by a New York University User on 06/22/2015
oropharynx. Indirect laryngoscopy re¬ vealed an epiglottis shifted to the right, with obliteration of the left aryepiglottic fold by a large, well-circumscribed, mobile mass. The left vocal cord was difficult to visualize due to obstruction of the vestibule. A computed tomographic scan of the neck demonstrated a tumor of the left supraglottic larynx consistent with a fluid-filled laryngoeele (Fig 1). The patient was taken to the operating room for excision of the mass. A nasotracheal tube was placed endoscopically, and direct laryngoscopy was performed. A large tumor was noted in the left supraglottic area, oc¬ cluding the laryngeal vestibule. The overly¬ ing mucosa appeared normal. Needle aspira¬ tion of the mass yielded only a small amount of mucoid material, which was submitted for cytologie analysis. A transverse incision was made in the skin, between the hyoid bone and the thyroid cartilage, and a 6-cm cystic tumor was then easily palpable beneath the strap muscles. The mass was dissected free of the surrounding tissues and the epiglottis to the level of the thyroid cartilage. The excised tumor was lobular and had the consistency of a mucocele. A frozen-section specimen was consistent with a lipoma. Gross examination revealed a 6.5 5.0 2.5-cm mass, with a thin, fibrous capsule and a mottled, nodular surface (Fig 2). On sec¬ tioning, the tumor was composed of grayishwhite, gelatinous material characteristic of myxoma. The diagnosis was confirmed on paraffin section (Fig 3). Postoperatively, the patient did well, with gradual lessening of his hoarseness. He re¬ mains well 12 months after his operation. or
Fig 1 Computed tomographic scan of myxoma of the supraglottic larynx. Left. Axial section at the level of the hyoid bone. Right. Lower axial section just above level of vocal cords. —
.
fibroxanthomas, liposarcomas, fibrous
dysplasia, or nodular fasciitis. Histologically, myxomas contain in¬ conspicuous stellate cells with bland '"''
nuclei, abundant mucoid material that stains
positive for mucopolysaccharides, and a rich network of delicate
reticular fibers."" Characteristics of a preclude the diagnosis of include the presence of myomyxoma blasts, lipoblasts, or chondroblasts.2 A myxoid lesion with evidence of malig¬ nancy is usually a sarcoma with areas of myxoid change, often a liposartumor that
Gross appearance of myxoma following
Fig 2.
resection. —
Fig 3. —Histologie section demonstrating both collagenous and myxomatous elements within the tumor (hematoxylin-eosin, 250).
coma."
histologie differential diagnosis laryngeal myxoma includes the myxoid type of vocal cord polyp, with which it is commonly mistaken. Un¬ like myxomas, vocal cord polyps dis¬ play prominent blood vessels and fre¬ quently feature subepithelial basement membrane hyalinization. Myxoid vocal cord polyps are always covered by stratified squamous epithelium, in con¬ The
of COMMENT The most common clinical presenta¬ tion of a myxoma is that of a slowly growing, painless mass.' Myxomas of the head and neck occur with equal frequency in men and women and tend to occur in the third and fourth de¬ cades of life, although individuals of all ages can be affected.L2J Babbitt and Pfeiffer" reported a myxoma of the palate in a 14-year-old female patient; our patient with a laryngeal myxoma was a
70-year-old
man.
The origin of myxomas is unknown, but antecedent infection or trauma has been proposed as initiating events.' Myxomas may be tumors of primitive
mesenchyme,
a
concept supported by
the gross and histologie similarities between the two tissues. The tumor contains delicate, interlacing reticular fibers and abundant mucopolysaccharide characteristic of primitive mesen¬ chyme.1' Myxomas of the mandible of¬ ten occur around erupting teeth and contain odontogenic epithelium; they may also arise from odontogenic, pri¬ mordial mesenchyme." Grossly, myxomas are gray, white, or opalescent, gelatinous tumors that may appear encapsulated due to com¬ pression of surrounding tissue.3 They are typically rubbery in texture, but can range from soft to firm in consis¬ tency, and often resemble fibromas,
a
trast to myxomas, which may or may not be lined with such epithelium. In
addition, myxoid vocal cord polyps al¬ ways
occur on
the true vocal cord.
Myxomas of the head and neck usu¬ ally arise in the mandible or maxilla, but they can also occur in the parotid gland, orbit, mucosa of the oral cavity and oropharynx, or soft tissues of the face and neck.121" The only other case of a laryngeal myxoma reported in the
Downloaded From: http://archotol.jamanetwork.com/ by a New York University User on 06/22/2015
English-language literature was that by Chen and Ballecer," in 1986. It occurred in a 37-year-old man who complained of dysphagia and dysphonia secondary to a pedunculated myxo¬ ma of the lingual epiglottis. The treatment of a myxoma depends on
its location and size. Due to their
encapsulated appearance, myxomas are often simply enucleated. The pseu¬ docapsule around the tumor is rarely complete, however, and local recur¬ rence may result, usually within the first 3 years.3 Myxomas of bone may have a particularly high rate of recur¬
Zimmerman and Dahlin7 re¬ ported a series of 20 patients with myxomas of the jaw: four of those patients had recurrent tumors on pre¬ sentation, and in four patients tumors recurred following the first or second excision. Ten patients underwent re¬ section of myxomas of the mandible or maxilla at Memorial Sloan-Kettering Cancer Center. Nine patients had their tumors excised with a portion of rence.
the
underlying bone,
recurrence
at 10
without tumor years. One local excision
or more
patient underwent a only, and in this patient
tumor
re¬
curred 5 years later.'" Successful resection of a myxoma of the facial bones mandates adequate exposure. Keller12 discusses the resec¬ tion of a myxoma of the maxilla by means of a Lefort I osteotomy. Petti and coworkers13 describe the resection of a mandibular myxoma using a sagit¬ tal ramus osteotomy. Myxomas of soft tissue are probably less likely to recur following resection.3 Ireland et al' reported only two recur¬ rences of tumors in 45 examined pa¬ tients.' Removing the tumor with a margin of normal tissue will minimize the risk of local recurrence; frozensection analysis of questionable mar¬ gins should be used to ensure adequate resection. Multiple myxomas of the head and neck that occur in a young patient should alert the clinician to the possi-
bility of a systemic disorder character¬ ized by cardiac- and soft-tissue myxo¬
(often of the head and neck), facial and labial skin pigmentation, and en¬ docrine overactivity (Cushing's syn¬
mas
drome, acromegaly).
"
Carney
et al15
series of 40 patients with this disorder, which appears to be in¬ herited in an autosomal dominant fash¬ ion. Of note is that 25% of the patients died of disease secondary to emboliza¬ tion from a cardiac myxoma. In summary, myxomas of the head and neck are uncommon lesions, with
reported
a
laryngeal tumors being particularly rare. Although benign, their propensi¬ ty for local recurrence mandates good intraoperative exposure and excision with a margin of normal tissue. When facial and labial pigmentation and/or signs of endocrine overactivity appear, the patient with a head and neck myx¬ oma should be examined for myxoma.
a
cardiac
References 1. Canalis RF, Smith GA, Konrad HR. Myxoof the head and neck. Arch Otolaryngol.
mas
1976;102:300-305. 2. Stout AP. Myxoma tumor of primitive mesenchyme. Ann Surg. 1948;127:706-719. 3. Batsakis JG. Myxomas of soft tissues and the facial skeleton. Ann
Otol Rhinol
Laryngol.
1987;96:618-619. 4. McClure DK, Dahlin DC. Myxoma of bone. Mayo Clin Proc. 1977;52:249-253. 5. Ireland DCR, Soule EH, Ivins JC. Myxoma of somatic soft tissues. Mayo Clin Proc. 1973;48:
401-410. 6. Chen KTK, Ballecer RA. Laryngeal myxoma. Am J Otolaryngol. 1986;7:58-59. 7. Zimmerman DC, Dahlin DC. Myxomatous tu-
of the jaws. Oral Surg Oral Med Oral Pathol. 1958;11:1069-1080. 8. Babbitt JA, Pfeiffer DB. Myxoma of the pal-
mors
ate and
pharynx.
Arch
Otolaryngol. 1937;26:
453-458. 9. Fu YS, Perzin KH. Non-epithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx: a clinico-pathologic study, VII: myxomas. Cancer. 1977;39:195-203. 10. Ghosh BC, Huvos AG, Gerold FP, Miller TR. Myxoma of the jaw bones. Cancer.
1973;31:237-240. 11. Robbins SL, Cotran RS, Kumar V. Clinical aspects of neoplasia. In: Pathologic Basis of Disease. 3rd ed. Philadelphia, Pa: WB Saunders Co; 1984:270.
Downloaded From: http://archotol.jamanetwork.com/ by a New York University User on 06/22/2015
12. Keller EE. Resection of a myxoma of the maxilla via Lefort I osteotomy. J Oral Maxillofac
Surg. 1988;46:609-613.
13. Petti NA, Weber FL, Miller MC. Resection of a mandibular myxoma via a sagittal ramus osteotomy. J Oral Maxillofac Surg. 1987;45:793-795. 14. Cook CA, Lund BA, Carney JA. Mucocutaneous pigmented spots and oral myxomas: the oral manifestations of the complex of myxomas, spotty pigmentation and endocrine overactivity. Oral Surg Oral Med Oral Pathol. 1987;63:175-183. 15. Carney JA, Hruska LS, Beauchamp GD, Gordon H. Dominant inheritance of the complex of myxomas, spotty pigmentation, and endocrine overactivity. Mayo Clin Proc. 1986;61:165-172.
