|Year : 2016 | Volume
| Issue : 1 | Page : 51-53
Hamartoma of parapharyngeal space: A rare case report
Swati Tandon, Ravi Meher, Anoop Raj, Chandala Chitguppi
Departments of Otolaryngology-Head and Neck Surgery, MAM College and Associated, LN Hospital, New Delhi, India
|Date of Web Publication||25-Jan-2016|
Departments of Otolaryngology-Head and Neck Surgery, MAM College and Associated, LN Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
A hamartoma is a tumor-like lesion made up of tissues indigenous to the part but lacks the true growth potential of a true neoplasm. Hamartomas are rare in head and neck region. Since they are often asymptomatic, they are easily missed during routine examination. To the best of our knowledge, lymphangiomatous hamartoma of parapharyngeal space has not been reported in medical literature so far. We report a rare case of lymphangiomatous hamartoma of parapharyngeal space in an 8-year-old child managed by surgery.
Keywords: Hamartoma, head and neck hamartoma, lymphangioma, parapharyngeal space
|How to cite this article:|
Tandon S, Meher R, Raj A, Chitguppi C. Hamartoma of parapharyngeal space: A rare case report. MAMC J Med Sci 2016;2:51-3
| Introduction|| |
Hamartoma is a nonneoplastic malformation due to inborn error of tissue development, often with an abnormal mixture of tissue indigenous to the region. A hamartoma may occur in any organ as it designates a focal overgrowth of mature normal cells and tissues at sites of identical cellular composition. The etiology of head and neck hamartomas is unclear. The clinical manifestation of hamartoma in the head and neck region is always the same as that of a benign tumor. Because of its low clinical morbidity and nonspecific symptoms, head and neck hamartomas remain a diagnostic challenge. We describe a rare case of lymphangiomatous hamartoma of parapharyngeal space managed by surgery.
| Case Report|| |
An 8-year-old male child presented to Otolaryngology Department of our hospital with complaints of swelling noticed by parents on the right side of neck 3 months back [Figure 1]. The swelling was approximately the size of lemon and was nonprogressive. It was not associated with pain, fever, or inflammation of overlying skin. There was no history of dysphagia, change in voice or respiratory distress. Child took treatment from a local traditional practitioner who tattooed the overlying skin, but there was no change in size of swelling.
On local examination of the neck, a 5 cm × 4 cm swelling was seen on the right side of the neck below the angle of mandible. It was nontender, soft to firm in consistency with overlying skin unremarkable except for the tattoo marks. Contrast-enhanced computed tomography (CT) neck revealed well-defined hypodense cystic lesion seen in the right carotid space behind the ipsilateral sternocleidomastoid muscle extending superiorly from C1 to inferiorly till C5 [Figure 2].
|Figure 2: Contrast-enhanced computed tomography of neck showing the extent of lesion|
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Magnetic resonance imaging (MRI) with magnetic resonance angiogram of neck was performed which revealed a large lobulated lesion measuring 4.3 cm × 3.9 cm in the right para- and retro-mandibular space with no definite feeders from major vessels of the neck [Figure 3]. Fine-needle aspiration cytology from the swelling revealed Frank hemorrhagic aspirate with the possibility of benign vascular lesion. Thus, a provisional diagnosis of parapharyngeal hemangioma was made.
Child was planned for excision of the swelling under general anesthesia. A horizontal skin incision was given over the swelling at the level of hyoid and subplatysmal flaps elevated. Intraoperatively, the swelling was found lying deep to sternocleidomastoid muscle, lateral to carotid sheath, abutting it, but no invasion was seen. The swelling had both solid and cystic components. A small feeder vessel was seen arising from common carotid artery supplying the swelling. It was cauterized and swelling was dissected from the surrounding tissue and removed in toto with careful preservation of spinal accessory and hypoglossal nerves. The histopathological examination of postoperative specimen revealed large cystic anastomosing lymphatic channels with adipose tissue, small blood vessels, and smooth muscle bundles haphazardly present in the lesion lymphangiomatous hamartoma [Figure 4].
| Discussion|| |
The term “hamartoma” was introduced by Albrecht in 1904 who distinguished between true neoplasms and tumor-like lesions. Hamartomas are considered to be benign tumor-like malformations or nonneoplastic developmental anomalies caused by endogenous tissues that are proliferating in a disorganized manner. It is composed of tissue elements normally found at the site of origin, but which are growing in a disorganized mass, and most cases are asymptomatic. Hamartomas are pathologically sub-classified, depending on the relative abundance of a particular endogenous tissue, and the variants described include (1) vascular, (2) muscle predominant, (3) adipose tissue predominant, and (4) intramuscular capillary variants where numerous thin-walled mature capillaries are interspersed between and around muscle bundles.
In a study by Wushou et al, on 194 cases of head and neck hamartomas, they found that hamartomas are more common in male patients and frequently occur in the first and second decades. The most common sites involved were the oral mucosa (68.6%) and skin (27.3%). In other areas, hamartomas of the nasal septum, buccal space, olfactory cleft, nasopharynx, parotid gland, and larynx have been described. A case of rhabdomyomatous hamartoma of lower lip causing trigeminal neuralgia has been described.
Microscopically, the lymphovascular hamartomas contain fibrocollagenous tissue, adipose tissue, skeletal muscle fibers, few nerve bundles, immature or primitive mesenchyme along with lymph channels, and blood vessels interspersed between them, as was seen in our case.
Preoperatively in hamartomas of head and neck, CT and MRI must be performed to know the extent of the lesion, any bony infiltration or intracranial involvement. In cases of vascular hamartomas, angiogram must be performed to know the vascularity of tumor and feeding vessel.
Hamartomas must be differentiated from teratomas, dermoid cysts, and choristomas by subjecting the complete specimen to histopathological examination.
Hamartomas have no capacity for continuous unimpeded growth, so their proliferation is self-limiting. They have no tendency to regress spontaneously either. Nevertheless, they are prone for recurrences especially when excised incompletely. As the clinical course of hamartoma is essentially benign, the treatment of choice is surgical excision. Complete removal of tumor mass by wide surgical excision is the ideal treatment. In the present patient also, complete surgical excision of the mass was performed with no recurrence at 6 months follow-up.
| Conclusion|| |
Hamartomas of head and neck are benign lesions. Lymphangiomatous hamartoma of parapharyngeal space are rare. They are usually asymptomatic and quite often missed in the clinics. Preoperative evaluation must include radiology to know the extent of lesion, and angiography to know for any feeder from great vessels in the neck. Complete surgical excision is the treatment of choice with good prognosis.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]