INTRODUCTION
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that comprises a
wide range of life-threatening superficial or infiltrative lesions. It has a
prevalence of 0.91 cases per 100,000 children, being most common in the
extremities. When it appears in the elderly, it has a greater malignancy chance
and may develop angiosarcoma or hemangiosarcoma. When it occurs in children, it
is benign1-3. The other histological differential
diagnoses of KHE include infantile hemangioma, congenital hemangioma,
fusocellular hemangioma, verrucous malformation/hemangioma and Kaposi’s
sarcoma1.
The microscopy examination is characterized by confluent nodules of
spindle-shaped neoplastic endothelial cells involving multiple planes of tissue
that are positive for endothelial, lymphatic, and smooth muscle markers2. The treatment approach for these tumors
is variable, mainly because the cases are rare, can be infiltrative and located
in an area of difficult surgical resection, and can also be related to the
Kasabach-Merritt phenomenon, characterized by an association of capillary
hemangioma and thrombocytopenia, which can cause bleeding, petechiae, bruises,
and spontaneous bruising4,5. Spontaneous tumor involution is rare; the
best treatment option is total excision. However, it is sometimes mutilating or
unviable, and other treatment options, use of corticosteroids, vincristine,
interferon, chemotherapy, embolization, propanolol, sclerotherapy, and
radiotherapy have been described2.
This article aims to describe the radiological correlation using resonance,
surgical and anatomopathological findings in a rare case of a child with
kaposiform hemangioendothelioma.
CASE REPORT
Boy admitted at the age of five in the Orthopedics Service of Hospital Sarah,
presenting a history of onset, at six months of age, of a tumor in the right
plantar region, painless, with wine aspect without inflammatory signs. He
underwent two surgical procedures for excision in another medical service, at
the age of two, with recurrence after three months, and at the age of four, with
recurrence afterward, having been diagnosed with plantar fibromatosis. When he
was admitted to this hospital, he had tumor recurrence in the plantar region and
difficulty to perform plantar support on the right and to wear shoes, due to
local pain. He presented a hardened tumor with a scar aspect on physical
examination, not painful on palpation, with an extension of about 5 cm in its
largest diameter, located in the right plantar region (Figure 1). AP and lateral plantar radiography and nuclear
magnetic resonance imaging showed a lesion in the posterior plantar region
measuring in the lateral-lateral direction, 3cm long x 2cm thick, respecting the
plantar fascia and directed to the skin. He had a normal blood count, platelet
count, and coagulogram, with no Kasabach-Merritt phenomenon. A biopsy was
indicated, whose report showed kaposiform cutaneous hemangioendothelioma.
Marginal tumor excision was performed, maintaining the fascia and primary suture
of the lesion measuring 3x2cm; he had a recurrence six months after the
operation. It was decided to enlarge the lateral surgical margin, and in-depth
by 2 cm, elliptical resection was performed, including skin, subcutaneous,
muscular fascia up to the calcaneus, measuring 5x3x2cm.
Figure 1 - Skin lesion in the right plantar region at admission and after
recurrence; (Right) MRI examination performed after relapse, showing
amorphous, heterogeneous, iso/hypointense tissue in T1, T2, and
STIR, extending deeply to the thickened plantar fascia plane with a
slight interval measuring about 5.7mm at its largest anteroposterior
axis, with the aforementioned amorphous tissue interposing. It
stands out in the most superficial subcutaneous tissue, immediately
before the surgical scar, an oval area with lobulated contours,
isointense in T1, and slightly hyperintense in T2 / STIR that
exhibited post-contrast enhancement. Measuring about 12.6mm in its
longest anteroposterior axis, 3.46mm in its longest lateral-lateral
axis, and 6.5mm without its longest craniocaudal axis, it suggested
recurrence.
Figure 1 - Skin lesion in the right plantar region at admission and after
recurrence; (Right) MRI examination performed after relapse, showing
amorphous, heterogeneous, iso/hypointense tissue in T1, T2, and
STIR, extending deeply to the thickened plantar fascia plane with a
slight interval measuring about 5.7mm at its largest anteroposterior
axis, with the aforementioned amorphous tissue interposing. It
stands out in the most superficial subcutaneous tissue, immediately
before the surgical scar, an oval area with lobulated contours,
isointense in T1, and slightly hyperintense in T2 / STIR that
exhibited post-contrast enhancement. Measuring about 12.6mm in its
longest anteroposterior axis, 3.46mm in its longest lateral-lateral
axis, and 6.5mm without its longest craniocaudal axis, it suggested
recurrence.
The intraoperative freezing exam showed fibrotic tissue, but with no evidence of
a tumor in the deep margin, with the excision of the plantar fascia, the
calcaneus periosteum, and reconstruction with flap rotation of the medial
pedicle plantar cavity measuring 6x4cm and skin grafting partial in the donor
region in the midfoot, 4-0 monocryl suture and Brown dressing in the grafted
area. The graft was removed with Blair’s knife from the medial portion of the
homolateral thigh; the dressing was maintained for a week when total skin graft
integration was observed. Rest with an elevated limb without load was instructed
until complete flap and skin graft integration for two weeks; the flap’s
satisfactory evolution with remission of pain was observed, without tumor
recurrence in the 15-year follow-up (Figure 2).
Figure 2 - A. Transoperative: tumor resection with wide lateral
margins and depth, dimensions of 5x3x3cm, up to the calcaneus.
Marking and dissection of the flap of the plantar cavity with medial
vascularization; B. Rotation of the flap measuring 6 cm
to cover the calcaneus; Partial skin graft removal from the medial
aspect of the homolateral thigh and distribution with skin graft and
4-0 monocryl suture; C. Dressing tied to the grafted
area; D. Postoperative result after 12 years of
follow-up.
Figure 2 - A. Transoperative: tumor resection with wide lateral
margins and depth, dimensions of 5x3x3cm, up to the calcaneus.
Marking and dissection of the flap of the plantar cavity with medial
vascularization; B. Rotation of the flap measuring 6 cm
to cover the calcaneus; Partial skin graft removal from the medial
aspect of the homolateral thigh and distribution with skin graft and
4-0 monocryl suture; C. Dressing tied to the grafted
area; D. Postoperative result after 12 years of
follow-up.
The anatomopathological examination revealed vascular neoplasia with infiltrative
lobes, separated by fibrous septa, containing compact, sometimes loose,
fusocellular areas, in capillary permeate, cracks, and vessels varying sizes,
covered by endothelium with a glomeruloid aspect, few figures of mitosis and
free surgical margins. Tumor karyotype (45, XY, -10 (3) / 46, XY (21) with
chromosome 10 monosomy (3 cells), normal clone (21 cells) culture time 8
days.
DISCUSSION
The natural history of kaposiform hemangioendothelioma, the differential
diagnosis, and treatment regime for these lesions remains unclear and challenges
plastic surgeons’ challenges. The authors describe the report of a rare case of
kaposiform hemangioendothelioma with a difficult diagnosis, which had previously
been operated on and relapsed three times. He presented a fibromatous aspect on
physical examination, leading to the clinical suspicion of plantar fibromatosis,
which was not confirmed by imaging and biopsy exams. In a biopsy,
histopathological findings, corroborating with the literature, were of confluent
nodules of spindle-shaped neoplastic endothelial cells involving multiple tissue
planes that were positive for smooth endothelial, lymphatic, and muscle markers;
this was important for the definition of the diagnosis.
In the first surgery for excision, the oncological criteria were respected,
preserving the fascia, following the NMR exam, which showed involvement in the
deep dermis, deep, subcutaneous, and muscular tissues; however, these parameters
were not sufficient for remission. of the tumor. After six months
postoperatively, there was a recurrence, and a new surgical approach was
performed, widening the lateral and deep margins, with the excision of the
fascia and periosteum, achieving remission during 15 years of follow-up without
important functional sequelae.
Kaposiform hemangioendothelioma tumor is a tumor of vascular origin according to
the classification of the International Society for the Study of Vascular
Anomalies (ISSVA), which covers all malformations and vascular tumors in an
internationally consistent nomenclature framework. It is one of two systems of
most used classification. It is based on Mulliken and Glowacki’s initial
classification in 19826 and has since been
updated with the recognition of causal genetic mutations; this classification
was revised in May 2018. Excision with a tumor-free surgical margin should be
the definitive treatment for KHE, although it is unattainable in some cases due
to the location, extent of the lesion, or association with coagulopathy. When
associated with coagulopathy, it can have a mortality rate of around 20%. Thus,
other treatment options are described, such as the use of corticosteroids,
single or combined chemotherapy, laser, and radiotherapy5-8.
Sirolimus®, also called rapamycin, is a drug used as an immunosuppressant.
It is a macrocyclic lactone produced by organisms of the species Streptomyces
hygroscopicus, a macrolide mTOR inhibitor with an antiangiogenic effect. It has
been reported as a successful agent in treating refractory and complicated cases
of KHE, reducing tumor, pain, and coagulopathy phenomena. However, there is no
single defined protocol, and its use in children with vascular anomalies is
limited. It was usually indicated when other alternatives failed.
The reconstruction of the plantar region, corroborating with other authors9-12 and as presented in the case report, was a challenge due to the
need to have the flap with sufficient dimensions to cover the defect and protect
the calcaneus, to have skin similar to the region, low volume and sufficient
sensitivity to prevent injuries, support load, avoid pain when walking and
enable the use of shoes. Among the available options are the rotating plantar
cavity’s local flaps, the medial plantar flap, the reverse sural flap, distance
flaps, and free flaps9-12. It was not possible to perform a skin
graft due to bone exposure. In this case report, it was possible to cover a 5 cm
defect in the largest axis located in the calcaneus region, using the plantar
cavity flap and partial skin grafting in the donor area. The flap and skin graft
showed good integration, which allowed for the functional recovery of the
foot.
CONCLUSION
In this report of the case of a child with kaposiform hemangioendothelioma, a
rare vascular tumor, it was not possible to make a clinical diagnosis at
admission. A biopsy and histopathological examination were necessary. In the NMR
exam, diffuse heterogeneous enhancement was identified after contrast of the
lesion involving the skin, from the subcutaneous tissue to the fascia. These
limits were used for resection up to the fascia, but they were not sufficient
for tumor control, and there was recurrence after six months. It was necessary
to re-approach the resection of the fascia and periosteum to control the
vascular lesion’s growth, which, although benign, has endothelial growth with an
invasion of the skin, muscle, fascia to the periosteum. The exeresis was
performed on the lateral margins and in-depth, up to the periosteum, and
extensive reconstruction with the plantar cavity flap and partial skin grafting
in the donor region, with no signs of recurrence after 15 years. We emphasize
the surgeon’s importance to go beyond the margins delimited by NMR for the
control of the disease.
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1. Rede Sarah de Hospitais, Plastic Surgery,
titular member of SBCP, Brasília, DF, Brazil.
2. Rede Sarah de Hospitais, Nursing, Brasília, DF,
Brazil.
3. UNICEUB, Medical Student, Brasília, DF,
Brazil.
4. Fundação de Ensino e Pesquisa em Ciências da
Saúde Escola Superior de Ciências da Saúde, Nursing, Brasília, DF,
Brazil.
*Corresponding author: Maria Ireni Zapalowski Galvão
SMHS Quadra 501, Bloco A, Asa Sul, DF, Brazil. Zip Code: 70335-970 E-mail:
201995@sarah.br
Article received: August 06, 2019.
Article accepted: January 10, 2021.
Conflicts of interest: none