Hemangiopericytoma
in the thenar region
(Portuguese
PDF version)
Alberto
Coimbra Duque¹, Manuel Julio Cota Janeiro²,
Ivanésio Merlo²
1.
Professor, Universidade Gama Filho; Professor, Pontifícia Universidade
Católica do Rio de Janeiro.
2.
Vascular Surgeon, Clínica do Aparelho Circulatório (Clinic
of Circulatory System).
Correspondence:
Dr. Alberto Coimbra Duque
Rua Sorocaba, 464/201
CEP 22271-110 - Rio de Janeiro - RJ
E-mail: acduque@uol.com.br
ABSTRACT
The
authors describe a pulsatile nodular lesion on the hand of a 51
year-old male. The lesion was diagnosed as hemangiopericytoma, a
rare vascular tumor. Clinical status, treatment and possible complications
of the hemangiopericytoma are discussed.
Key
words: hemangiopericytoma, tumor, hand.
Palavras-chave: hemangiopericitoma, tumor, mão.
J
Vasc Br 2003;2(1):23-25
INTRODUCTION
M.R.F.,
a white, married, 51-year-old Brazilian man, presented with pulsatile,
oval nodule on the palm of the left hand. According to the patient,
who was followed up during ten months, the nodule had not been causing
him any problem, but four weeks before he sought medical care, the tumor
became sore and was associated with paresthesia of the hand.
The clinical, cardiological and laboratory examinations yielded normal
results. Examination of the site showed a solid and oval-shaped tumor
growth, about 2 cm in diameter, with tactile sensation of pulse on the
left palm, in the thumb region. The Doppler flow meter revealed intense
triphasic arterial flow, suggestive of arteriovenous fistula at the
site, although other fistula-related complications, such as venous engorgement,
continuous murmur or skin disorders, were not observed.
The color flow duplex scanning confirmed the presence of tumor growth
in the thenar region, showing a mesh of arteries and veins at the center,
with no signs of arteriovenous fistula (Figure 1).
 Figure
1 - Color flow duplex scanning of the hemangiopericytoma.
Magnetic
resonance showed a clearly defined oval-shaped lesion to the soft parts
of the thenar region, about 1.5 cm in size, extending to the first metacarpophalangeal
joint, resembling a giant cell tumor of the synovial sheath.
We opted for tumor excision. The patient was submitted to surgery under
regional anesthesia, with sedation and without the use of Esmarch bandage.
The round-shaped nodular mass was totally resected, with feeder vein
and arteries, which were properly ligated. The mass was located next
to nerves and tendons, but had not affected them.
The histopathological examination showed a well-demarcated, circumscribed
lesion, containing irregular vascular structures, many of which were
slit-shaped, limited by flat cells and separated by abundant stroma
formed by elongated cells with spindle-shaped nuclei of regular size,
without atypical cells. Around the lesion were Pacinian corpuscles,
nerve filaments and medium-sized and small blood vessels. Hemangiopericytoma
of the left hand was diagnosed (Figures 2 and 3). The healing occurred
without greater complications, with preservation of hand function (Figure
4). Two years after tumor excision, the patient remains asymptomatic.
Figure
2 - Aspect of the surgical specimen.
Figure
3 - Enlarged image of the tumor showing proliferation of pericytes.
Figure
4 - Patient's hand after tumor excision.
DISCUSSION
Hemangiopericytoma
is a very rare vascular tumor, containing endothelial tubes enveloped
in round or elongated cells, which supposedly derive from pericytes, as
the epithelioid cells in a glomus tumor.1 Pericytes
are contractile cells that sheathe capillary vessels and that work by
changing the diameter of the vessel lumen.2
In most cases, the tumors developed in pericytes are of benign nature
and their growth does not cause systemic complications to the patient;
however, local malignant invasion, in addition to distance metastasis
have already been described.3
The favorable outcome, in most cases, seems to result from the properties
of pericytes which sometimes behave like precursor cells, and give rise
to fibroblasts and endothelial cells. Some cases present hypoglycemia
due to the secretion of substances with insulin-like properties by the
tumor.4
Literature data show that tumors with over 6.5 cm in diameter and foci
of necrosis, hemorrhage, and elevated mitotic index usually indicate malignancy.5,6
Metastases often occur hematogenously, but they may also affect the lymph
nodes. In these cases, a careful palpation of lymph nodes is important
and, if some suspicion exists, an excision should be performed to collect
material for histopathological analysis.
In the case reported herein, the outcome was benign. Contact with the
patient two years after resection confirmed that he is well, been working
and has shown no symptoms of the disease. We emphasize the importance
of early resection in these cases.
ACKNOWLEDGMENTS
Special
thanks to professor and Dr. Anadil Roselli for the histopathological analysis.
REFERENCES
1.
Anderson WAD, Scotti TM. Sinopse de Patologia. Rio de Janeiro: Cultura
Moderna; 1970.
2.
Stout AP. Hemangiopericitomas. Câncer 1949;2(1):217-20.
3.
Murad TM, von Haam E, Murthy MS. Ultrastructure of a hemangiopericitoma
and glomus tumor. Cancer 1968;22(6):239-49.
4.
Fabris VE. Tumores Vasculares. In: Maffei FHA, editor. Doenças
Vasculares Periféricas. Rio de Janeiro: Medi; 1995.
5.
Kaufman SL, Stout AP. Hemangiopericytoma in children. Cancer 1960;13(1):695.
6.
McMaster MJ, Soule EH, Ivins JC. Hemangiopericytoma. A clinicopathologic
study and long-term followup of 60 patients. Cancer 1975;36(6):2232-44.
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