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Glandular hamartoma of the larynx
International Journal of Pediatric Otorhinolaryngology 65 (2002) 163– 166 www.elsevier.com/locate/ijporl
Case report
Glandular hamartoma of the larynx O. Gazi Yigitbasi a,*, Gulen Guler b, Figen Ozturk c, Ercihan Guney d a
Erciyes Uni6ersity Medical Faculty, Otorhinolaryngology and Head & Neck Surgery Department, Kayseri, Turkey b Erciyes Uni6ersity Medical Faculty, Anesthesiology Department, Kayseri, Turkey c Erciyes Uni6ersity Medical Faculty, Pathology Department, Kayseri, Turkey d Erciyes Uni6ersity Medical Faculty, Otorhinolaryngology and Head and Neck Surgery Department, Kayseri, Turkey Received 22 February 2002; received in revised form 16 May 2002; accepted 20 May 2002
Abstract Glandular hamartoma of the larynx is an extremely rare lesion, and the number of well-documented and acceptable cases is limited. Presenting symptoms may include changes in voice, eating and activity levels, and respiratory complaints. We report on a 14-month-old infant with this rare clinical entity. Direct laryngoscopy revealed a well-mucosalized, encapsulated, firm, 0.5 cm wide and 2.5 cm long lesion that originated from the right aryepiglottic fold and reached into the nasopharynx. The mass was completely excised endoscopically. Histopathological examination revealed a hamartoma, which was composed of glandular elements, mixed with mesodermal tissues. After endoscopic removal of the hamartoma, the child was relieved of the obstruction. © 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Hamartoma; Larynx
1. Introduction Hamartoma is a developmental anomaly characterized by the formation of a tumor-like mass composed of mature tissue elements that are normally present in the location where it is found, but occurring in abnormal proportions or arrangement [1,2]. Localization in the head and neck region is uncommon. However hamartomas have occasionally been described in the sinonasal tract, * Corresponding author. Present address: Millet Cad. Belediye Durmaz Is Hani, Kat. 3, No. 305, Kayseri, Turkey. Fax: + 90-3524-379-164 E-mail address: [email protected] (O.G. Yigitbasi).
nasopharynx, oral cavity, oropharynx, larynx, hypopharynx, cervical esophagus, ear, parotid gland, trachea, parathyroid gland, and eye [3]. Hamartoma of the larynx is an extremely rare lesion, and the number of well-documented and acceptable cases is limited. Signs and symptoms vary, but there is generally some degree of voice change and upper airway obstruction [4]. The pathology may be misleading and can often be confused with other benign tumors of the larynx. Management should consist of conservative excision, and endoscopic removal is usually adequate. Larger lesions are approached with open techniques, and the extent of the tumor dictates the degree of excision [5]. Recurrences are usually
0165-5876/02/$ - see front matter © 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 2 ) 0 0 1 5 0 - 7
164
O.G. Yigitbasi et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 163–166
associated with incomplete removal; the prognosis is good in completely excised lesions [3]. The aim of this report is to remind pediatricians, anesthesiologists and pediatric otorhinolaryngologists that this rare congenital abnormality has to be taken into account.
2. Case report A 14-month-old boy was referred to our clinic with stridor and nourishment difficulties. Routine examination of the oral cavity revealed a 0.5 cm wide, easily moving mass on both sides, covered by normal-appearing mucosa, which reached into nasopharynx and extended into the laryngopharynx (Fig. 1). A pectus excavatum deformity was also revealed in systemic examination. There were no additional pathologic findings in radiographic studies of the chest. It was decided to take the patient to operation for the management of stridor and nourishment difficulties. For anesthesia, 5 mg/kg of thiopentone, 10 mg/kg of atropine and 1 mg/kg of succinylcholine were administered to the patient via an intravenous catheter. A mobile mass, extending into the larynx, was noticed during laryngoscopy. For intubation, the mass was pulled out of the larynx with the help of a clamp. The patient received 2– 3% sevoflurane and 50%
Fig. 1. Routine examination of oral cavity revealed a 0.5 cm wide, easily moving mass on both sides, covered by normal-appearing mucosa which reached into nasopharynx and lied down into laryngopharynx.
Fig. 2. Microlaryngoscopic examination under general anesthesia revealed soft, pulsatile and well-mucosalized mass, which originated from the right aryepiglottic fold just over the arytenoid cartilage and its free end reached into nasopharynx.
nitrous oxide in oxygen for the maintenance of anesthesia. Microlaryngoscopic examination under general anesthesia revealed a soft, pulsatile and well-mucosalized mass, which originated from right aryepiglottic fold just over the arytenoid cartilage; its free end reached into the nasopharynx (Fig. 2). The mass was entirely extracted from its origin with microlaryngeal forceps (Fig. 3). The base of the lesion arose from the right aryepiglottic fold just over the arytenoid
Fig. 3. Well-mucosalized, encapsulated, firm 0.5 cm wide and 2.5 cm long lesion that originated from the right aryepiglottic fold was entirely extracted from its origin with microlaryngeal forceps.
O.G. Yigitbasi et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 163–166
165
the larynx (Figs. 4 and 5). Later at 42 months, the patient has no evidence of recurrence clinically.
3. Discussion
Fig. 4. The microscopic appearance of the lesion is poorly organized glands and smooth muscle, nerve trunks, fibroblastic elements (H and E, original 200 × ).
cartilage. Extubation was performed without any difficulty. The patient was kept under care for 2 days in our department without upper airway symptoms or nourishment difficulties. Histopathologic examination revealed a polypoid lesion covered by acanthotic squamous epithelium. The mass was composed of well-organized smooth muscle fibers admixed with mucous glands and occasional large nerve trunks. There were also spindle shaped fibroblasts and collagenous tissue. The stroma showed mild chronic inflammation. There was no evidence of malignancy. The final pathologic diagnosis was glandular hamartoma of
Fig. 5. Microscopically polyp was composed of well-oriented smooth muscle fibers admixed with mucous glands, and nerve trunks, fibroblastic elements covered by epithelium (H and E, original 100 × ).
In younger patients, congenital abnormalities including teratomas, dermoid cysts, and choristomas rank high in the differential diagnosis. Teratomas contain mature or immature tissues derived from all three germ layers— endoderm, mesoderm, and ectoderm. Dermoid cysts are composed of stratified squamous epithelium, with ectodermal and mesodermal elements that form dermal appendage-like structures; they frequently enlarge by accumulating squamous debris. A choristoma is an abnormal accumulation of mature tissue at on anatomic site in which that tissue is not usually found. In contrast, a hamartoma is a localized proliferation of mature cells and tissues in the region where they are normally found, but arranged in an architecturally abnormal pattern [6]. In 1904, Albert [7] introduced the term hamartoma to describe a tumor-like malformation composed of tissue elements native to the organ in which they arise. Hamartoma designates an excessive focal overgrowth of mature normal cells and tissues in an organ composed of identical cellular elements [5]. Hamartomas described in the literature fall into two broad categories. The first and more common group consists of mesenchymal hamartomas composed of an overgrowth of mesodermal tissues without any epithelial component (e.g. lipoma, chondroma) [8]. The second less common type consists of the epithelial or glandular hamartomas, composed of epithelial or glandular elements mixed with mesodermal tissues (e.g. fibrous tissue, cartilage). The present report reviews a glandular type of hamartoma in the larynx. The diagnosis is made by histological examination of an excised specimen. The treatment of laryngeal hamartomas consists of conservative surgical excision. Because of the well-encapsulated nature and small size of these lesions, endoscopic removal is usually adequate, as in this case. There does not seem to be
166
O.G. Yigitbasi et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 163–166
any advantage in the use of a carbon dioxide laser in comparison with conventional techniques. Larger lesions are approached with open techniques [6]. In conclusion, hamartomas are rare lesions in the head and neck. The most common site is the nasopharynx, and even in that region, only five well-documented cases have been reported in which both stromal elements and ducts or glands are present. We report a glandular hamartoma originating from the right aryepiglottic fold. Conservative excision with endoscopic approach has resulted in the cure of the patient.
[2]
[3]
[4] [5]
[6]
[7]
References [1] World Health Organization, Histological Typing of Tu-
[8]
mours of the Upper Respiratory Tract and Ear, vol. 1, Springer-Verlag, Berlin, Germany, 1991, p. 73. A. Ferlito, K.O. Devaney, Developmental lesions of the head and neck: terminology and biologic behaviour, Ann. Otol. Rhinol. Laryngol. 104 (1995) 913 – 918. A. Rinaldo, C. Fisher, G.M. Mannara, A. Ferlito, Hamartoma of the larynx: a critical review of the literature, Ann. Otol. Rhinol. Laryngol. 107 (1998) 264 – 267. S.R. Cohen, Posterior cleft larynx associated with hamartoma, Ann. Otol. Rhinol. Laryngol. 93 (1984) 443 – 446. S.M. Archer, D.M. Crockett, T.J.I. McGill, Hamartoma of the larynx: report of two cases and review of the literature, Int. J. Pediatr. Otorhinolaryngol. 16 (1988) 237 – 243. E.D. Fine, B. Dahms, J.E. Arnold, Laryngeal hamartoma: a rare congenital abnormality, Ann. Otol. Rhinol. Laryngol. 104 (1995) 87 – 89. E. Albert, Ueber hamartome, Verh. Dtsch. Ges. Pathol. 7 (1904) 153 – 157. J. Weinberger, O. Kassim, B.D. Birt, Hamartoma of the larynx, J. Otolaryngol. 14 (1985) 305 – 308.
Case report
Glandular hamartoma of the larynx O. Gazi Yigitbasi a,*, Gulen Guler b, Figen Ozturk c, Ercihan Guney d a
Erciyes Uni6ersity Medical Faculty, Otorhinolaryngology and Head & Neck Surgery Department, Kayseri, Turkey b Erciyes Uni6ersity Medical Faculty, Anesthesiology Department, Kayseri, Turkey c Erciyes Uni6ersity Medical Faculty, Pathology Department, Kayseri, Turkey d Erciyes Uni6ersity Medical Faculty, Otorhinolaryngology and Head and Neck Surgery Department, Kayseri, Turkey Received 22 February 2002; received in revised form 16 May 2002; accepted 20 May 2002
Abstract Glandular hamartoma of the larynx is an extremely rare lesion, and the number of well-documented and acceptable cases is limited. Presenting symptoms may include changes in voice, eating and activity levels, and respiratory complaints. We report on a 14-month-old infant with this rare clinical entity. Direct laryngoscopy revealed a well-mucosalized, encapsulated, firm, 0.5 cm wide and 2.5 cm long lesion that originated from the right aryepiglottic fold and reached into the nasopharynx. The mass was completely excised endoscopically. Histopathological examination revealed a hamartoma, which was composed of glandular elements, mixed with mesodermal tissues. After endoscopic removal of the hamartoma, the child was relieved of the obstruction. © 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Hamartoma; Larynx
1. Introduction Hamartoma is a developmental anomaly characterized by the formation of a tumor-like mass composed of mature tissue elements that are normally present in the location where it is found, but occurring in abnormal proportions or arrangement [1,2]. Localization in the head and neck region is uncommon. However hamartomas have occasionally been described in the sinonasal tract, * Corresponding author. Present address: Millet Cad. Belediye Durmaz Is Hani, Kat. 3, No. 305, Kayseri, Turkey. Fax: + 90-3524-379-164 E-mail address: [email protected] (O.G. Yigitbasi).
nasopharynx, oral cavity, oropharynx, larynx, hypopharynx, cervical esophagus, ear, parotid gland, trachea, parathyroid gland, and eye [3]. Hamartoma of the larynx is an extremely rare lesion, and the number of well-documented and acceptable cases is limited. Signs and symptoms vary, but there is generally some degree of voice change and upper airway obstruction [4]. The pathology may be misleading and can often be confused with other benign tumors of the larynx. Management should consist of conservative excision, and endoscopic removal is usually adequate. Larger lesions are approached with open techniques, and the extent of the tumor dictates the degree of excision [5]. Recurrences are usually
0165-5876/02/$ - see front matter © 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 2 ) 0 0 1 5 0 - 7
164
O.G. Yigitbasi et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 163–166
associated with incomplete removal; the prognosis is good in completely excised lesions [3]. The aim of this report is to remind pediatricians, anesthesiologists and pediatric otorhinolaryngologists that this rare congenital abnormality has to be taken into account.
2. Case report A 14-month-old boy was referred to our clinic with stridor and nourishment difficulties. Routine examination of the oral cavity revealed a 0.5 cm wide, easily moving mass on both sides, covered by normal-appearing mucosa, which reached into nasopharynx and extended into the laryngopharynx (Fig. 1). A pectus excavatum deformity was also revealed in systemic examination. There were no additional pathologic findings in radiographic studies of the chest. It was decided to take the patient to operation for the management of stridor and nourishment difficulties. For anesthesia, 5 mg/kg of thiopentone, 10 mg/kg of atropine and 1 mg/kg of succinylcholine were administered to the patient via an intravenous catheter. A mobile mass, extending into the larynx, was noticed during laryngoscopy. For intubation, the mass was pulled out of the larynx with the help of a clamp. The patient received 2– 3% sevoflurane and 50%
Fig. 1. Routine examination of oral cavity revealed a 0.5 cm wide, easily moving mass on both sides, covered by normal-appearing mucosa which reached into nasopharynx and lied down into laryngopharynx.
Fig. 2. Microlaryngoscopic examination under general anesthesia revealed soft, pulsatile and well-mucosalized mass, which originated from the right aryepiglottic fold just over the arytenoid cartilage and its free end reached into nasopharynx.
nitrous oxide in oxygen for the maintenance of anesthesia. Microlaryngoscopic examination under general anesthesia revealed a soft, pulsatile and well-mucosalized mass, which originated from right aryepiglottic fold just over the arytenoid cartilage; its free end reached into the nasopharynx (Fig. 2). The mass was entirely extracted from its origin with microlaryngeal forceps (Fig. 3). The base of the lesion arose from the right aryepiglottic fold just over the arytenoid
Fig. 3. Well-mucosalized, encapsulated, firm 0.5 cm wide and 2.5 cm long lesion that originated from the right aryepiglottic fold was entirely extracted from its origin with microlaryngeal forceps.
O.G. Yigitbasi et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 163–166
165
the larynx (Figs. 4 and 5). Later at 42 months, the patient has no evidence of recurrence clinically.
3. Discussion
Fig. 4. The microscopic appearance of the lesion is poorly organized glands and smooth muscle, nerve trunks, fibroblastic elements (H and E, original 200 × ).
cartilage. Extubation was performed without any difficulty. The patient was kept under care for 2 days in our department without upper airway symptoms or nourishment difficulties. Histopathologic examination revealed a polypoid lesion covered by acanthotic squamous epithelium. The mass was composed of well-organized smooth muscle fibers admixed with mucous glands and occasional large nerve trunks. There were also spindle shaped fibroblasts and collagenous tissue. The stroma showed mild chronic inflammation. There was no evidence of malignancy. The final pathologic diagnosis was glandular hamartoma of
Fig. 5. Microscopically polyp was composed of well-oriented smooth muscle fibers admixed with mucous glands, and nerve trunks, fibroblastic elements covered by epithelium (H and E, original 100 × ).
In younger patients, congenital abnormalities including teratomas, dermoid cysts, and choristomas rank high in the differential diagnosis. Teratomas contain mature or immature tissues derived from all three germ layers— endoderm, mesoderm, and ectoderm. Dermoid cysts are composed of stratified squamous epithelium, with ectodermal and mesodermal elements that form dermal appendage-like structures; they frequently enlarge by accumulating squamous debris. A choristoma is an abnormal accumulation of mature tissue at on anatomic site in which that tissue is not usually found. In contrast, a hamartoma is a localized proliferation of mature cells and tissues in the region where they are normally found, but arranged in an architecturally abnormal pattern [6]. In 1904, Albert [7] introduced the term hamartoma to describe a tumor-like malformation composed of tissue elements native to the organ in which they arise. Hamartoma designates an excessive focal overgrowth of mature normal cells and tissues in an organ composed of identical cellular elements [5]. Hamartomas described in the literature fall into two broad categories. The first and more common group consists of mesenchymal hamartomas composed of an overgrowth of mesodermal tissues without any epithelial component (e.g. lipoma, chondroma) [8]. The second less common type consists of the epithelial or glandular hamartomas, composed of epithelial or glandular elements mixed with mesodermal tissues (e.g. fibrous tissue, cartilage). The present report reviews a glandular type of hamartoma in the larynx. The diagnosis is made by histological examination of an excised specimen. The treatment of laryngeal hamartomas consists of conservative surgical excision. Because of the well-encapsulated nature and small size of these lesions, endoscopic removal is usually adequate, as in this case. There does not seem to be
166
O.G. Yigitbasi et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 163–166
any advantage in the use of a carbon dioxide laser in comparison with conventional techniques. Larger lesions are approached with open techniques [6]. In conclusion, hamartomas are rare lesions in the head and neck. The most common site is the nasopharynx, and even in that region, only five well-documented cases have been reported in which both stromal elements and ducts or glands are present. We report a glandular hamartoma originating from the right aryepiglottic fold. Conservative excision with endoscopic approach has resulted in the cure of the patient.
[2]
[3]
[4] [5]
[6]
[7]
References [1] World Health Organization, Histological Typing of Tu-
[8]
mours of the Upper Respiratory Tract and Ear, vol. 1, Springer-Verlag, Berlin, Germany, 1991, p. 73. A. Ferlito, K.O. Devaney, Developmental lesions of the head and neck: terminology and biologic behaviour, Ann. Otol. Rhinol. Laryngol. 104 (1995) 913 – 918. A. Rinaldo, C. Fisher, G.M. Mannara, A. Ferlito, Hamartoma of the larynx: a critical review of the literature, Ann. Otol. Rhinol. Laryngol. 107 (1998) 264 – 267. S.R. Cohen, Posterior cleft larynx associated with hamartoma, Ann. Otol. Rhinol. Laryngol. 93 (1984) 443 – 446. S.M. Archer, D.M. Crockett, T.J.I. McGill, Hamartoma of the larynx: report of two cases and review of the literature, Int. J. Pediatr. Otorhinolaryngol. 16 (1988) 237 – 243. E.D. Fine, B. Dahms, J.E. Arnold, Laryngeal hamartoma: a rare congenital abnormality, Ann. Otol. Rhinol. Laryngol. 104 (1995) 87 – 89. E. Albert, Ueber hamartome, Verh. Dtsch. Ges. Pathol. 7 (1904) 153 – 157. J. Weinberger, O. Kassim, B.D. Birt, Hamartoma of the larynx, J. Otolaryngol. 14 (1985) 305 – 308.