Oral neurovascular hamartoma: An extraordinary verdict in the oral cavity

Abstract

The presence of a neurovascular hamartoma within the oral cavity is truly a rare entity. Scarcely reported in the literature, these hamartomas are smooth, pinkish masses and are painless, and therefore difficult to diagnose. They are benign in nature and apply pressure to their surroundings. The histological diagnosis remains the gold standard as it comprises of neural tissue and vascular components. Treatment is surgical excision with adequate margins. Recurrence is reported in cases of incomplete resection.

Full text

CASE REPORT
Oral neurovascular hamartoma: an extraordinary
verdict in the oral cavity
Montasir Junaid,
1
Sadaf Qadeer Ahmed,
2
Maliha Kazi,
2
Saroona Haroon
3
1
Department of
Otorhinolaryngology-Head and
Neck Surgery, Jinnah Medical
and Dental College, Karachi,
Sindh, Pakistan
2
Department of
Otorhinolaryngology-Head and
Neck Surgery, Aga Khan
University Hospital, Karachi,
Sindh, Pakistan
3
Department of
Histopathology, Aga Khan
University Hospital, Karachi,
Sindh, Pakistan
Correspondence to
Dr Maliha Kazi,
maliha_kazi@hotmail.com
Accepted 6 June 2014
To cite: Junaid M,
Ahmed SQ, Kazi M, et al.
BMJ Case Rep Published
online: [please include Day
Month Year] doi:10.1136/
bcr-2013-200832
SUMMARY
The presence of a neurovascular hamartoma within the
oral cavity is truly a rare entity. Scarcely reported in the
literature, these hamartomas are smooth, pinkish masses
and are painless, and therefore difficult to diagnose.
They are benign in nature and apply pressure to their
surroundings. The histological diagnosis remains the gold
standard as it comprises of neural tissue and vascular
components. Treatment is surgical excision with
adequate margins. Recurrence is reported in cases of
incomplete resection.
BACKGROUND
The term hamartoma is derived from the Greek
word ‘hamartion’which literally means bodily
defect. It was initially used by Albrecht in 1904.
1
It
refers to a benign malformation in different areas
of the body where growth occurs. The histology
consists of an abnormal mixture of cells and tissues
localised at the particular site secondary to a devel-
opmental error. They do not develop as part of an
inflammatory or neoplastic process and have a self
limiting proliferation.
2
On the other hand, they do
not have a tendency to regress spontaneously.
3
While considering the oral cavity, the hamartoma
might result secondary to the proliferation of smooth
muscles, skeletal muscles, fat, salivary tissues, vessels
and lymphatics.
4
An oral neurovascular hamartoma is
rare because of proliferation of neural tissue solely or
in combination with vascular elements.
Described below is an unusual case of an oral
hamartoma with neurovascular components.
CASE PRESENTATION
A 14-year-old boy presented to the outpatient
department with right cheek swelling for the past
1.5 years. The swelling had been gradually increas-
ing over a period of time and eventually became
impossible to ignore. He also reported of decreased
mouth opening and a burning sensation within the
oral cavity on eating spicy food for 2 years. He did
have a significant history of consuming betel nut
since the age of 11 years, discontinuing the use
once the mouth opening started reducing. His right
cheek also had decreased sensation, but there were
no symptoms of fever or pain.
The rest of the history was unremarkable.
On examination, the boy appeared to be physic-
ally fit with average height and weight. He had a
diffuse 2×1.5 cm sized right cheek swelling about
two finger breadths below the zygoma. The swel-
ling was soft in consistency, non-tender, non-mobile
and non-translucent. The swelling would become
firm on clenching of teeth and mobile on bimanual
examination.
Examination of the oral cavity showed significant
submucous fibrosis with poor oral hygiene and full-
ness in the right buccal mucosa. Neck examination
was unremarkable as was the systemic examination.
As part of his workup, his complete blood picture
was normal. The patient brought with him a CT
scan of poor quality and thick cuts. The lesion was
not clearly outlined but owing to financial constraint
the scan was not repeated (figure 1). No lymphaden-
opathy was identified on the scan, however.
The family was counselled and an excisional
biopsy of the lesion was performed.
INVESTIGATIONS
Histological findings confirmed the diagnosis. The
tissue specimen showed haphazard distribution of
mature skeletal muscles of varying sizes and nerve
fibres closely intertwined with each other on H&E
staining (figure 2). The specimen was also evalu-
ated with immunehistochemical stains, which
showed positive reactivity with S-100 (figure 3).
The lesion was concluded to be neurovascular
hamartoma of the oral cavity.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis of the disease included
infectious and malignant causes including traumatic
hamartoma.
Figure 1 CT image of a lesion in the right masseter
muscle.
Junaid M, et al.BMJ Case Rep 2014. doi:10.1136/bcr-2013-200832 1
Novel treatment (new drug/intervention; established drug/procedure in new situation)

TREATMENT
Performing a 2 cm incision over the right buccal mucosa, below
the Stenson’s duct, the dissection was continued using dissection
scissors. On reaching the masseter muscle and locating the mass
adjacent to the ramus of the mandible, the mass was removed
with wide margins (figure 4). The cavity was closed with proper
tissue reapproximation using absorbable sutures. Postoperatively,
the patient was provided with ice packs to prevent extensive
swelling and started on intravenous augmentin 1.2 g/q8h and
analgesics for 48 h after which he was switched to oral augmen-
tin in a dose of 625 mg thrice a day and oral analgesics.
He was discharged on the second postoperative day and
advised a soft diet for the next 3 days.
OUTCOME AND FOLLOW-UP
On follow-up a week later he was pain-free with the wound
healing well.
He was counselled regarding the disease and recommended a
six to eight monthly follow-up.
Reviewed in the outpatient department a year later, the boy
was symptom and disease free on clinical examination.
DISCUSSION
Hamartomas arising within the oral cavity are unusual and may
show a variety of clinical presentations and histological and
growth patterns. The biological behaviour is benign with
reasons for development vague. While epithelial and mesenchy-
mal hamartomas involving the oral cavity are rare, one cannot
deny that the presence of a neural or a neurovascular compo-
nent in a hamartoma is even more infrequent.
45
The literature
has only reported a handful of these cases.
In 2007, Takeyama et al
5
reported of a hamartoma on the
hard palate associated with corpus callosum agenesis, micro-
phthalmia and skin malformation. Prior to that, Goldsmith
et al
6
described a case of leiomyomatous hamartoma of the pos-
terior tongue leading to dysphagia in a 16-year-old, whereas
Miyamoto et al
7
documented a lingual hamartoma in a child
with a cleft palate. Al Habeeb et al
8
reported a solitary but cuta-
neous neural hamartoma in a middle-aged individual. The
lesion had histological characteristics comprising of numerous
mature nerve bundles within the papillary dermis. Prior to that,
a case of congenital neural hamartoma on the leg was reported
by Argenyi et al.
9
Vascular hamartomas are most common of all
in the head and neck.
10
As well as being sporadic, hamartomas can present as a part
of a syndrome. Epithelial hamartomas can be a part of cron-
khite–Canada syndrome or orofacial digital syndrome,
11
whereas neuromuscular and vascular hamartomas may become
apparent in Crohn’s disease.
12
Oral neurovascular hamartomas present as smooth surfaced
exophytic lesions usually less than 1 cm in diameter. Being ped-
unculated or wide based, their colour may range from pink to
yellow. They are painless lesions leading to general discomfort
in the patient.
Histologically, there is no clear boundary between the lesions
and the surrounding normal structures as they are non-
encapsulated and poorly circumscribed. Composed of hypocel-
lular connective tissue containing aggregates of loose vessels
closely packed with small to medium sized nerve bundles, they
are not easily distinguishable from the surrounding non-
hamartomatous tissues. These nerve bundles are reactive with
S-100 protein. This feature can be easily used as a criterion for
diagnosis while examining.
13
This reactivity was also pivotal in
our case to reach the final diagnosis. Distinction from quite a
few oral benign lesions is still hard. Oral neurovascular hamarto-
mas resemble traumatic hamartomas and can be distinguished
Figure 3 Immunohistochemical stain of the same, showing positive
staining for S-100.
Figure 2 High power magnification of the tissue with H&E staining,
showing interwoven mature skeletal muscles and nerve fibres.
Figure 4 Excision of the mass using an intraoral incision.
2 Junaid M, et al.BMJ Case Rep 2014. doi:10.1136/bcr-2013-200832
Novel treatment (new drug/intervention; established drug/procedure in new situation)

based on the fact that the blood vessels in the former are very
closely entwined with the neural component. In traumatic neur-
omas the two components are separate.
4
Once the diagnosis is confirmed the clinical management
comprises of surgical excision. Local excision is curative; a man-
agement plan similar to most benign oral lesions. A recurrence
only follows an incomplete excision and one should not mistake
it with malignant transformation.
14
Close clinical follow-up is
absolutely essential. The literature does not say much regarding
local recurrence of oral neurovascular hamartomas but oral mes-
enchymal hamartomas arising from the cheek have a reported
local recurrence rate of 20%.
15
CONCLUSION
Although oral neurovascular hamartomas are rare entities, they
should be considered in the differential diagnosis of oral lesions.
It is essential, therefore, to know their clinical presentation and
treatment plan.
Learning points
▸Oral neurovascular hamartoma is a well-recognised entity.
▸Knowledge regarding its diagnosis, histological findings and
treatment should be at hand despite it being a rare
condition.
▸Surgical excision cures the disease and recurrence is only in
cases of incomplete resection.
Contributors MJ operated on this patient and wrote the first draft; SQ reviewed
the draft of the case report; MK reviewed the case report and finalised the
manuscript; SH helped in writing the histopathological details for the case report.
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES
1 Edgerton MT. The treatment of hemangiomas with special reference to the role of
steroid therapy. Ann Surg 1976;183:517–32.
2 Carranza MA, Martin MC, Borrego H. Lingual hamartoma, report of a case. Acta
Otorhinolaryngol 2000;51:749–50.
3 Eichel BS, Hallberg OE. Hamartoma of the middle ear and eustachian tube: report
of case. Laryngoscope 1996;76:1810–15.
4 Kreiger PA, Ernst LM, Elden LM, et al. Hamartomatous tongue lesions in children.
Am J Surg Pathol 2007;31:1186–90.
5 Takeyama J, Miyota H, Nagasaka H. Hamartoma on the hard palate associated with
corpus callosum agenesis, microphthalmia, and skin malformation. J Oral Maxillofac
Surg 2007;65:556–9.
6 Goldsmith P, Soames JV, Meikle D. Leiomyomatous hamartoma of the posterior
tongue: a case report. J Laryngol Otol 1995;109:1190–1.
7 Miyamoto Y, Nagayama M, Hayashi Y. A cleft palate child with lobulated tongue
and lingual hamartoma: report of a case. J Oral Maxillofac Surg 1991;49:644–6.
8 Al Habeeb A, Alkhalidi H, Idikio H, et al. Cutaneous solitary neural hamartoma:
report of an unusual case. Am J Dermatopathol 2009;31:484–6.
9 Argenyi ZB, Goodenberger ME, Strauss JS. Congenital neural hamartoma (fascicular
schwannoma). A light microscopic, immuno-histochemical, and ultra-structural
study. Am J Dermatopathol 1990;12:283–93.
10 Endo R, Matsuda H, Takahashi M, et al. Respiratory epithelial adenomatoid
hamartoma in the nasal cavity. Acta Otolaryngol 2002;122:398–400.
11 Chen HM, Fang JY. Genetics of the hamartomatous polyposis syndromes:
a molecular review. Int J Colorectal Dis 2009;24:865–74.
12 Hemmings CT. Neuromuscular and vascular hamartoma arising in a Meckel’s
diverticulum. Pathology 2006;38:173–4.
13 Allon I, Allon DM, Hirshberg A, et al. Oral neurovascular hamartoma: a lesion
searching for a name. J Oral Pathol Med 2012;41:348–53.
14 Mahindra S, Daljit R, Sohail MA, et al. Hamartomas of the nose. J Laryngol Otol
1978;92:57–60.
15 LeBer MS, Stout AP. Benign mesenchymoma in children. Cancer 1962;15:598–605.
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Novel treatment (new drug/intervention; established drug/procedure in new situation)