E. Cigna; B. Carlesimo; G. Bistoni; F. Conte; F. Palumbo; N. Scuderi
University of Rome “La Sapienza”, Department of Dermatology and Plastic Surgery, Rome, Italy
Vyšlo v časopise:
ACTA CHIRURGIAE PLASTICAE, 50, 2, 2008, pp. 55-58
tumors are benign hamartomas arising from the glomus apparatus and
are located in the subcutaneous tissue. These tumors are caused by
proliferation of glomus cells, which make up a portion of the
glomus body (1, 5, 8, 10, 11, 14).
normal glomus body is a contractile neuromyoarterial receptor
comprising an afferent arteriole, an anastomotic Sequet-Hoyer canal,
an efferent venule, actin-containing glomus cells, the
intraglomerular reticulum, and a capsular portion that
contributes to the regulation of subcutaneous tissue temperature (1,
glomus tumors are thought to arise from glomus cells, they have been
observed in extracutaneous locations not known to contain glomus
cells. Two variants exist: solitary glomus tumors and multiple glomus
tumors, also known as glomangiomas (1, 5, 8, 10, 14).
initiating event for glomus cell proliferation is still unknown. Some
authors have postulated that trauma induces solitary subungual glomus
tumors, although this theory has yet to be confirmed (1, 10, 11, 14).
Multiple lesions are slightly more common in males. Multiple glomus
tumors, particularly those of the disseminated variant, are inherited
in an autosomal-dominant pattern with incomplete penetrance (1). Most
hereditary glomangiomas are associated with defects in the glomulin
gene, located on chromosome 1 (1, 5, 8, 10, 11).
diagnosis of glomus tumors is generally clinical. However, several
imaging techniques are still being used to support the clinical
diagnosis before undergoing surgical treatment (1, 11).
instrumental tests used to confirm the clinical evaluation may
include: X-ray, MRI, arteriography and ultrasound, with or without
Doppler analysis (2, 3, 4, 6, 13).
the glomus tumor is common and includes pain, pinpoint pain,
hypersensitivity to cold, a bluish or purple color to the
finger, and papules or nodules that blanch on pressure.
therapy for glomus tumors is surgical excision, with a high rate
of success (2, 3, 4, 6).
review of our treated cases, here we report our assessment scale that
simplifies the diagnosis of this neoplasm and may, in our opinion,
avoid unnecessary expensive radiological examinations.
1997 to 2007, 21 patients were diagnosed and surgically treated for
solitary glomus tumor of the digit at the Department of Plastic,
Reconstructive and Aesthetic Surgery of the University of Rome “La
patient population comprised of 19 females and 2 males. The
mean age at diagnosis was 35.5 years (range 19 to 53 years). All
patients had a single glomus tumor located in the distal phalanx
of the right hand. Patients were divided in two groups.
I included nine most recent cases treated surgically after
clinical diagnosis alone using our method (Table 1).
II included twelve cases previously treated and retrospectively
analyzed and compared to Group I. The analysis of Group II was
done using recording charts (Table 2).
but two cases resolved with
a single operation. Two patients had undergone two operations
for incomplete excision of the lesion before being admitted to our
Department. Before our clinical examination, the patients in Group II
had undergone numerous invasive and extensive instrumentaltests. On ten patients, 18 imaging procedures
were performed: 4 X-rays, 7 ultrasound, 4 MRI and 3 angiography (see
excision of the nodule was performed under local anesthesia with
mepivacaine 1% digital block on digital ischemia by finger
tourniquet. The excision of the sensitive afferent nervous branch was
performed under loupe magnification (2.5 X to 4 X) in order to
visualize the afferent nervous branch.
histological appearance of the glomus tumor is a solid neoplasm
composed of groups of monomorphic glomus cells with large circular
nuclei and a small amount of cytoplasm.
the center of the neoplasm are well-circumscribed vascular spaces
delimited by endothelium surrounded by a vallum
of fibrous tissue that is compressed and dislocated by the mass.
There are three distinct histologic types of glomus tumor, a mucoid
hyaline, a solid, and an angiomatous type.
the different types of glomus tumors, immunohistochemistry revealed
a positive presence to alfa-actin and alfa-caldesin and
negativity to CD-34, S-100, and CK tests.
review of our treated cases, the symptoms in the two groups were
classified according to frequency and clinical relevance to
diagnosis. Major symptoms were the most frequent and the most
specific, whereas minor symptoms were less frequent and less specific
(see Table 1).
symptoms included paroxysmal pain caused by glomus-cell contraction,
pinpoint pain and hypersensitivity to cold. Minor symptoms included
blue or purple color of the digit, papules and nodules that blanched
upon pressure (see Table 1). On review of the case histories, minor
symptoms appeared to be related to the time of tumor onset, appearing
years after the start of the first symptoms that were usually
paroxysmal pain and hypersensitivity to cold.
treated patients had complete regression of pain after surgical
excision of the tumor, except for one patient who had hypoesthesia of
the treated area for three months after surgery. All patients were
examined at follow-up at 6 months and 1 year by cold-sensitivity
test and pinpoint test (Love test) and had a negative response.
patients had a hypertrophic scar that was treated with silicone
gel for 2 to 6 months.
tumors, also known as glomic tumors, glomangiomas, or
hemangiopericytomas are benign neoplasms smaller than one centimeter
in diameter and commonly located in the distal extremities,
particularly in subungual areas. This tumor represents from 1 to 5%
of all soft tissue tumors of the hand (1, 5, 7, 8, 10, 11).
there are several symptoms and signs that have been reported to be
related to this neoplasm. Three clinical tests are used to identify
the glomus tumor. The Love test consists of eliciting pain by
applying pressure to a precise area with the tip of a pencil.
The Hildreth sign eradicates pain after application of a tourniquet
proximally on the arm. The cold-sensitivity test should result in
increased pain when the affected region is exposed to cold
temperature (7, 11). However, none of these tests were considered
specific to accurately diagnose the tumor. For this reason patients
undergo imaging studies for the possible occurrence of glomus tumor
prior to surgery.
the physical examination the differential diagnosis considered other
tumors that appeared to be glomus tumors. Schwannoma or neurilemmoma
are tumors that most frequently mimic the clinical presentation of
a glomus tumor. However, they are not usually painful and do not
cause hypersensitivity to cold. Other conditions that must be
considered in differential diagnosis for hand tumors may include
hemangiomas, leiomyomas, angiolipomas, mucous cysts, neurofibromas,
Raynaud phenomenon, and osteogenic tumors (1, 5, 11).
imaging studies have been used to define the radiological aspect of
the lesion and facilitate differential diagnosis. In the past we used
these studies to confirm the diagnosis before surgical excision,
particularly when clinical evaluation was not adequate to achieve
a correct diagnosis.
showed bone erosion in 14-60% of patients and an increased distance
between the dorsum of the phalanx and the underside of the nail.
However, X-rays are not highly repeatable, due to the
ionizing power, and more importantly are not very accurate (11).
scanning images may show a hypoechoic-nodular lesion compared to
the surrounding fat, which is usually marked off by a regular,
thin hyperechoic vallum. This test is accurate, low-cost,
non-invasive, non-ionizing and highly repeatable. Ultrasound can also
be performed with the power-Doppler mode to assess the blood-flow
study (2, 3, 4, 6). The ultrasound power-Doppler has been used to
study the artero-venous shunt, a typical characteristic of the
glomus tumor, and has now replaced arteriography in the study of
vascular morphology. The vascular study is effective in confirming
the diagnosis but does not add information that can facilitate
surgical excision (2, 3, 4, 6). In fact, arteriography is used also
for an accurate study of the vascular composition of the glomus
tumor, but this method is invasive, poorly tolerated by patients, and
has a low repeatability level, due to its invasiveness and
ionizing power (2, 3, 4, 6).
magnetic-resonance imaging (MRI) has proved to be a valuable
method for imaging glomus tumors and can be useful in efficiently
identifying preoperative tumors, particularly those with non-specific
symptoms. However, MRI has poor sensitivity to diagnose this type of
tumor and is an expensive diagnostic test (1, 11).
our opinion clinical examination and diagnosis should never replace
an instrumental test. A negative clinical examination may
influence the surgical approach while the opposite condition will
preferred treatment for solitary glomus tumors is surgical excision
that is effective and curative. The prognosis is excellent with
complete regression of pain after surgery (9, 11).
our experience, we believe that clinical examination of the patient
is usually sufficient to diagnose glomus tumor. With respect to the
nine cases we recently treated, and after review of previously
treated cases, it is our opinion that two major symptoms, or one
major plus two minor symptoms, are adequate to achieve a high
accuracy rate in diagnosing glomus tumor. However, in selected cases
with uncertain symptoms or clinical signs or multi-operated patients
for recurrence due to inadequate excision of the glomus tumor,
ultrasound completed by Doppler ultrasonography may support the
diagnosis by the surgeon of subungual glomus tumors. As a result,
we achieved a total accuracy of 90% (19/21 patients), 100% on
the most recent cases (9/9 patients), and of 82% (9/11 patients) on
previously operated cases even though the negative cases were
analyzed in a retrospective study of the case histories.
Federico Barocci, 3
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