World Journal of Pharmaceutical Research
Doddawad et al.
Volume 5, Issue 12, 342-347.
SJIF Impact Factor 6.805
World Journal of Pharmaceutical
Research
Review Article
ISSN 2277– 7105
HAMARTOMA OF HEAD AND NECK REGION: A REVIEW
1
*Dr. Vidya G. Doddawad, 2 Dr. Shivananda S., 3 Dr. Kavita B. Gaddikeri, 4 Dr.
Chandrakala J., 5 Dr. Girish K.L. and 6 Dr. Sunita S.
1
*Reader Department of Oral Pathology and Microbiology JSS Dental College and Hospital
Mysore.
2
Reader Department of Oral Surgery JSS Dental College and Hospital Mysore.
3
4
Reader Department of Oral Pathology and Microbiology Bidar.
Reader Department of Oral Pathology and Microbiology Government Dental College and
Hospital Bangalore.
5
Professor Department of Oral Pathology and Microbiology Sree Mookambika Institute of
Dental Science Kulasekheram.
6
Reader Department of Public Health Dentistry JSS Dental College and Hospital Mysore.
ABSTRACT
Article Received on
09 Oct. 2016,
Hamartoma is an uncommon lesion/mass in head and neck region, that
Revised on 29 Oct. 2016,
Accepted on 19 Nov. 2016
shows a variety of clinical presentations, histological appearances and
DOI: 10.20959/wjpr201612-7451
growth
pattern.
Hamartoma
is
a
non-neoplastic
developmental
anomaly that appears as simple spontaneous growths, composed of a
*Corresponding Author
mixture of locally derived mature tissue which does not destroy the
Dr. Vidya G. Doddawad
adjacent tissue. Although hamartoma is not a true neoplasm, but it
Reader Department of Oral
behaves like tumour and it recurs if the tumour excised incompletely.
Pathology and Microbiology
JSS Dental College and
KEYWORDS:
Hospital Mysore.
incompletely.
Hamartoma
is
an
uncommon
region,
that
INTRODUCTION
Hamartoma as defined in the Webster's medical dictionary as 'a mass resembling a tumour
that represents anomalous development of tissue natural to part or an organ rather than a true
tumour'. They grow along with and at the same rate as the parent tissue unlike a cancerous
tumor. The hamartoma word is derived from Greek word ‘hamartia’ means 'scribal error or
mistake'.[1] Hamartoma is an excessive focal proliferation or overgrowth of cells or tissue
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which is native to its origin. Therefore, it has been believed that these lesions are
developmental aberrations.
History
The term 'hamartoma' was introduced by Albrecht in 1904 to describe an inborn error of
tissue development characterized by an abnormal mixture of tissues indigenous to the part,
with excess of one or more. The tumour-like malformation grows with the body until
maturity of the tissues is attained – unlike tumours which are autonomous new growths[1] and
ceases to reproduce, so the growth is self-limiting. It is non-neoplastic malformation or
inborn error in the development of one of the three germinal layers.[2] Hamartomas may occur
anywhere in the body, but are encountered most frequently in the lungs, kidney and liver and
rare in head and neck region.[3] Development of multiple hamartoma is known as
hamartomatosis.[4] Hamartoma is grow along with and at the same rate as the organ from
whose tissue they are made and unlike cancerous tumors, only rarely invade or compress
surrounding structures significantly.[5]
In 1967, Willis further elucidated the term 'hamartoma'
and he has extensively discussed
different hamartomas common to a specific germinal layer, which had created confusion and
controversy over the years. Hamartomas are tumour-like malformations due to an anatomical
error in the course of development of the body to its adult state.[3] They grow concurrently
with the host forming a mass of recognizable tissue which contains the structures derived
from any of the three germinal layers. These must be differentiated from teratoma, dermoids
and choristoma.
The teratoma implies a spontaneous autonomous new growth derived from pluripotential
tissue which is composed of elements of all three germinal layers. For an example: Ovarian
teratoma. In earlier literature, tertoma and hamartomas were included under the common
name teratoids (Eichel and Hallberg 1966). Dermoids have the cystic space and same
histogenesis as teratoma which originate from two layers, the ectoderm and mesoderm.
Another concept is choriostoma which is neoplasm of ectopic tissue i.e excessive
proliferation of tissue which are not native to its particular location. For an example: gingival
salivary gland, lingual thyroid. Clinically it is very difficult to differentiate between
teratomas, dermoids, choriostomas and hamartomas.[2]
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In reviewing the literature, we found that hamartomas are unusual in the head and neck
region and many cases were reported and are often seen in infancy and childhood.[1] Ishii et
al. reviewed cases of hamartoma of the tongue, Eichel and Hallberg S reported a case of
hamartoma of the middle ear, Ladap reported a case of nasopharyngeal hamartoma, Ravi et al
reported a case of hamartoma at the infratemporal fossa [1] and 12 cases occurring in the neck
were described by Lattes and Pachter. Samuel et al reported a case of hamartoma of the
ethmoid sinus and also in the parotid gland region.[3] Hamartomas have been described in the
nasal cavity, oropharynx, retropharyngeal, hypopharynx, larynx, ear, eustachian tube and the
deep neck spaces.[6,7] Intracranial lesions like the extra-cerebral glioneural hamartoma
extending into the para-pharyngeal space have also been described.[1]
Classification
1. Hamartomas can be classified into (a) mesodermal component containing type that is
more frequent and (b) the epithelial or glandular element containing type that is less
frequent.[1]
2. Working classification by Regazi and Weiss SW[5]
1. Odontogenic hamartoma
A. Those involving teeth: Dens invaginatus
Dens evaginatus
Talons cusp
Enamaloma
B. Those not involving teeth: Odontoma
Giagantiform cementoma
Dental lamina cyst of the new born
Adenomatoid odontogenic tumor
Squamous odontogenic tumor
Peripheral odontogenic tumor
Ameloblastic fibro-odontoma
2. Non-odontogenic tumor
A. Epithelial origin: Epsten pearl and Bohns nodule
Melanotic macule
Pigmented cellular nevus
B. Vascular origin: Haemangioma
Lymphangioma
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Glomus tumor
C. Osseous origin: Torus Platinus
Torus mandibularis
3. Unknown/doubtful origin
a. Granular cell myoblastoma
b. Congenital tumor of new born
c. Melanotic neuroectodermal tumor of infancy
d. Fibromatosis of gingiva
4. Syndrome associated with hamartoma
a. Cowden syndrome
b. Maffucci syndrome
c. Peutz jegher syndrome
d. Sturge weber angiomatosis
e. Klippel trenaunoy syndrome
3. Two histological types are recognized: the hyaline vascular type, characterized by small
follicles rich in blood vessels and the less common plasma cell type, which shows
vascular interfollicular tissue heavily infiltrated with plasma cells. [3]
4. Classification by Burkitz[9]
Hemangioma
Lymphangioma
Glomus tumor
Nevi
Granular cell tumor of the tongue
Granular cell epulis
Melanotic neuroectodermal tumor of infancy
Nerve sheath tumor
Traumatic neuroma
Fibrous dysplasia
Cherubism
Odontoma
Ossifying fibroma
Aneurismal bone cyst
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Traumatic bone cyst
Static bone cyst
Behaviour
In spite of not being a neoplasm in a true sense, the clinicopathological behaviour of a
hamartoma is like that of a neoplasm and it does not have tendency to regress spontaneously.
Nevertheless they are prone for recurrences especially when excised incompletely. They
rarely invade or compress adjacent structures significantly. Hamartomas have no capacity for
continuous unimpeded growth, so their proliferation is self limiting. This mixed bag of
characteristics has disputed the neoplastic origin of this uncommon lesion. Some authors
believe that the hamartomas are benign neoplasm rather than just malformations.[8,10]
Symptoms caused by hamartomas of the head and neck are not pathognomonic and it may
produce pressure symptoms in respective region, even though they may not actually invade
the structures.[8]
Hamartoma are usually congenital and have their major period of growth during growth of
body and it will reach adult dimension. They do not extend to involve more tissue and rarely
increase in size unless trauma, thrombosis, infection cause edema, inflammatory infiltration,
and filling of new vascular channels. It is found in many tissue of the body and a tendency to
such malformation is often hereditary.[9]
Oral hamartomas, are occur in association with other gross and microscopic developmental
abnormalities that assist considerably in their diagnosis.[9] A review of literature indicates that
epithelial and mesenchymal hamartoma are uncommon in the head and neck region.
CONCLUSION
As though, the hamartomas are clinically benign and histologically native to its origin, the
treatment of choice is surgical excision. There are no chance of recurrence if the tumour is
complete removed with wide surgical excision.
REFERENCE
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Challenge in the Management of a Rare Tumor of Skull Base and Face. International
Journal of Head and Neck Surgery, September-December 2011; 2(3): 148-150.
2. RK Jain. Hamartoma of the head and neck. Acta Medica Iranica, 1995; 33: 106-9.
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3. J Samuel, CCM Fernandes. Hamartomas of the head and neck. A report of 4 cases. S Air
Med J, 1985; 68: 265-267.
4. Juan Rosai. Rosai and Ackerman surgical pathology, 9 th ed. Vol.2.
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7. John C Tiffee, E Leon Barnes. Neuromuscular Hamartomas of the Head and Neck. Arch
Otolaryngol Head Neck Surg. 1998; 124(2): 212-216.
8. K Vikram Bhat, D Manjunath, N Srinivasan. Recurrent Lymphovascular Hamartoma of
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9. Greenberg MS, Glick M, Ship JA. Burkit’s oral medicine. 11 th edition. BC Decker Inc,
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10. Regazzi JA, Sciubba JJ. Connective tissue lesions oral pathology: clinicopathological
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