The Journal of Dermatology Vol. 18: 286-290, 1991
Diffuse Neonatal Hemangiomatosis with a Giant Cutaneous Hemangioma on the Axilla Koji Sayama, Takashi Higaki*, Hisashi Ohtsuka, Yuko Kobayashi, Yuji Shirakata, Satoshi Shiraishi and Yoshiharu Miki Abstract A case of diffuse neonatal hemangiomatosis is described. At birth, a giant tumor, 10 em in diameter and 6 em in height, was present on the left axilla and associated with thrombocytopenia. Numerous cherry-red papules were present on the skin and buccal mucosa. On the second day of life, the giant tumor was excised. Histological examination of the tissue revealed masses of endothelial cells embedded in fibrous tissues. Plain and enhanced computed tomography of the brain revealed a hemorrhage and two hemangiomas. The associations of intravascular papillary endothelial hyperplasia were discussed.
Key words: congenital multiple hemangiomatosis; thrombocytopenia; intravascular papillary endothelial hyperplasia
Introduction In congenital multiple hemangiomatosis (CMH), multiple small strawberry-type hemangiomas develop during gestation or within the first few weeks of life. Sometimes, systemic hemangiomas are found in three or more organ systems, in which cases they are called diffuse neonatal hemangiomatosis (1). In cases of extensive systemic hemangiomas, mortality can be high, mostly due to high-output cardiac failure often induced by arterio-venous shunts in the liver or lung. Thrombocytopenia and central nervous system involvement are also frequent (2). When no hemangiomas are found other than in the skin, the condition is called benign neonatal hemangiomatosis (3). In this report, a case of diffuse neonatal hemangiomatosis with a giant cutaneous hemangioma and thrombocytopenia is described. Received December 7, 1990; accepted for publication March 27, 1991. Department of Dermatology and *Department of Pediatrics, University of Ehime School of Medicine, Ehime 791-02,Japan. Reprint requests to: Koji Sayama, M.D., Department of Dermatology, University of Ehime School of Medicine, Shitsukawa, Shigenobu-cho, Onsen-gun, Ehime 791-02, Japan.
Fig. 1. A giant tumor on the left axilla present at birth.
Case Report The patient was a 2770 g infant of 39 weeks gestation, delivered by Caesarean section because an echogram had revealed a giant mass on the left chest wall at 36 weeks gestation. There was no family history of hemangiomatous syndrome. At birth, a giant pulsating tumor, 10 em in diameter and 6 em in height, was present on the left axilla (Fig. 1). A systolic bruit was noted on the tumor. Numerous cherry-red papules, less than 2 mm in diameter, were present on the trunk, extremities, face and buccal mucosa. The Apgar score was 7 and
Diffuse Neonatal Hemangiomatosis rose to 9 in 5 min. Initial laboratory studies showed a platelet count of 6.5 x 104 / pI. Serum glutamic oxaloacetic transaminase was 21 IU/mI. Serum fibrinogen was decreased to 23 ng/dl. Fibrin/fibrinogen degradation product (FDP) of the serum was increased to 32.7pg/ml. Urinalysis was normal, and stool Guaiac test was negative. Chest Roentgenograms showed compression of the left lung by the tumor (Fig. 2). An echogram of the tumor revealed pulsating vessels. No space-occupying lesions were demonstrated by echograms of the head and abdomen. The patient was treated with freshly frozen plasma and antithrombin III. However, the platelet count fell to 3.3 x 104 / pI when the tumor was excised on the second day oflife. Blood loss during the operation amounted to approximately 1,400 ml. Three weeks later, the platelet count and fibrinogen levels were within the normal range. FDP levels returned to normal in 3 months. Histological examination of the axillary tumor revealed masses of endothelial cells containing numerous small lumina or irregularly connected channels embedded in considerable amounts of fibrous tissue (Fig. 3A, B). Some lumina were irregularly dilated and were lined by a single layer of endothelial cells. Thrombi were found in some dilated luminal spaces. The cherry red papules showed a similar mass of endothelial cells in the upper dermis. Weigert staining of the tumor revealed some arteries and veins. However, no elements of a vascular wall, including elastic lamina, were identified at the periphery of the masses of endothelial cells or the irregularly dilated luminal spaces. Plain computed tomography (CT) of the brain revealed a high density area indicating a hemorrhage (arrow head, Fig. 4A), and contrast CT showed an enhanced area indicating a hemangioma (arrow head, 4B) at 3 months of age. In total, two hemangiomas were found. The hemorrhage was absorbed by 3 months. No neurological signs due to the hemorrhage or the hemangiomas were noted. At one year of age, the number and size of the cherry-red papules were still increasing (Fig. 5).
Comment No giant hemangioma like that found in the present case has been reported in CMH. One
287
Fig. 2. A chest Roentgenogram at birth. The left lung was compressed by the tumor.
case with a similar giant hemangioma on the axilla was reported without the association of CMH (4). In the present case, the giant hemangioma histologically consisted of masses of endothelial cells embedded within fibrous tissue, indicating that the tumor belonged to CMH. The systolic bruit and the echographic findings of the tumor indicated an arteriovenous shunt; however, angiography was not performed. This giant hemangioma, associated with thrombocytopenia and multiple hemangiomas in the skin, buccal mucosa, and brain, indicated that the present case was a type of diffuse neonatal hemangiomatosis. On the other hand, the histological findings, including the irregularly dilated luminal spaces lined with endothelial cells, the masses of endothelial cells with numerous small lumina, and the thrombi, may indicate an association with intravascular papillary endothelial hyperplasia (IPEH). However, no structures of the
288
Sayama et al
Fig. 3A. Photomicrograph of the axillary tumor. Masses of endothelial cells are embedded in fibrous tissues. Some lumina are irregularly dilated and are lined by a single layer of endothelial cells. (Hematoxylin-eosin, original magnification x20).
vascular wall were identified. The association with IPEH and the considerable amount of fibrous tissue may be attributed to repeated hemorrhage and thrombosis within the tumor,
which may also have contributed to the growth of the tumor. Platelet and fibrinogen consumption ceased after tumor resection, suggesting that the consumption took place within the
Diffuse Neonatal Hemangiomatosis
289
Fig. 3B. Photomicrograph of the axillary tumor. Higher magnification of the mass of endothelial cells. (original magnification x200).
tumor. The rate of involvement of the brain has been reported to be 52% in 25 cases of diffuse neonatal hemangiomatosis; it is often asso-
dated with seizures or hydrocephalus (5), and the mortality is high, more than 50% (5, 6). In the pr~sent case, the largest hemangioma could be excised, and the hemangiomas in the brain
Sayama et al
290
Fig. 4. Computed tomography (CT) of the brain at 3 months of age. A: plain CT. The high density area (arrow head) indicates a hemorrhage. B: contrast CT. The enhanced area (arrow head) indicates a hemangioma.
have not been large or frequent enough to induce neurological signs. These factors may contribute to the prognosis.
References 1) Holden KR, Alexander F: Diffuse neonatal hemangiomatosis, Pediatrics, 46: 411-421, 1970. 2) Artherton DJ, Rook A: Naevi and other developmental defects, in Rook A, Wilkinson DS, Ebling FJG, et al (eds): Textbook of Dermatology, Blackwell, London, 1986, pp 207-208. 3) Stem JK, Wolf JE Jr, Jarratt M: Benign neonatal hemangiomatosis,] Am Acad Dermatol; 4: 442-445, 1981. 4) Lofland GK, Filston HC: Giant cutaneous heman-
Fig. 5. Numerous cherry-red papules on the trunk at one year of age. gioma associated with axillary arteriovenous fistula causing congestive heart failure in the newborn infant,] PediatrSurg, 22: 458-460, 1987. 5) Golitz LE, Rudikoff'], O'Meara OP: Diffuse neonatal hemangiomatosis, PediatrDermatol, 3: 145-152, 1986. 6) Kennedy eTC, Fleming P, Duncan A: Eruptive cutaneous and visceral neonatal baemangiomatosis, Br] Dermatol, 123 (Suppl): 83-85, 1990.
Diffuse Neonatal Hemangiomatosis with a Giant Cutaneous Hemangioma on the Axilla Koji Sayama, Takashi Higaki*, Hisashi Ohtsuka, Yuko Kobayashi, Yuji Shirakata, Satoshi Shiraishi and Yoshiharu Miki Abstract A case of diffuse neonatal hemangiomatosis is described. At birth, a giant tumor, 10 em in diameter and 6 em in height, was present on the left axilla and associated with thrombocytopenia. Numerous cherry-red papules were present on the skin and buccal mucosa. On the second day of life, the giant tumor was excised. Histological examination of the tissue revealed masses of endothelial cells embedded in fibrous tissues. Plain and enhanced computed tomography of the brain revealed a hemorrhage and two hemangiomas. The associations of intravascular papillary endothelial hyperplasia were discussed.
Key words: congenital multiple hemangiomatosis; thrombocytopenia; intravascular papillary endothelial hyperplasia
Introduction In congenital multiple hemangiomatosis (CMH), multiple small strawberry-type hemangiomas develop during gestation or within the first few weeks of life. Sometimes, systemic hemangiomas are found in three or more organ systems, in which cases they are called diffuse neonatal hemangiomatosis (1). In cases of extensive systemic hemangiomas, mortality can be high, mostly due to high-output cardiac failure often induced by arterio-venous shunts in the liver or lung. Thrombocytopenia and central nervous system involvement are also frequent (2). When no hemangiomas are found other than in the skin, the condition is called benign neonatal hemangiomatosis (3). In this report, a case of diffuse neonatal hemangiomatosis with a giant cutaneous hemangioma and thrombocytopenia is described. Received December 7, 1990; accepted for publication March 27, 1991. Department of Dermatology and *Department of Pediatrics, University of Ehime School of Medicine, Ehime 791-02,Japan. Reprint requests to: Koji Sayama, M.D., Department of Dermatology, University of Ehime School of Medicine, Shitsukawa, Shigenobu-cho, Onsen-gun, Ehime 791-02, Japan.
Fig. 1. A giant tumor on the left axilla present at birth.
Case Report The patient was a 2770 g infant of 39 weeks gestation, delivered by Caesarean section because an echogram had revealed a giant mass on the left chest wall at 36 weeks gestation. There was no family history of hemangiomatous syndrome. At birth, a giant pulsating tumor, 10 em in diameter and 6 em in height, was present on the left axilla (Fig. 1). A systolic bruit was noted on the tumor. Numerous cherry-red papules, less than 2 mm in diameter, were present on the trunk, extremities, face and buccal mucosa. The Apgar score was 7 and
Diffuse Neonatal Hemangiomatosis rose to 9 in 5 min. Initial laboratory studies showed a platelet count of 6.5 x 104 / pI. Serum glutamic oxaloacetic transaminase was 21 IU/mI. Serum fibrinogen was decreased to 23 ng/dl. Fibrin/fibrinogen degradation product (FDP) of the serum was increased to 32.7pg/ml. Urinalysis was normal, and stool Guaiac test was negative. Chest Roentgenograms showed compression of the left lung by the tumor (Fig. 2). An echogram of the tumor revealed pulsating vessels. No space-occupying lesions were demonstrated by echograms of the head and abdomen. The patient was treated with freshly frozen plasma and antithrombin III. However, the platelet count fell to 3.3 x 104 / pI when the tumor was excised on the second day oflife. Blood loss during the operation amounted to approximately 1,400 ml. Three weeks later, the platelet count and fibrinogen levels were within the normal range. FDP levels returned to normal in 3 months. Histological examination of the axillary tumor revealed masses of endothelial cells containing numerous small lumina or irregularly connected channels embedded in considerable amounts of fibrous tissue (Fig. 3A, B). Some lumina were irregularly dilated and were lined by a single layer of endothelial cells. Thrombi were found in some dilated luminal spaces. The cherry red papules showed a similar mass of endothelial cells in the upper dermis. Weigert staining of the tumor revealed some arteries and veins. However, no elements of a vascular wall, including elastic lamina, were identified at the periphery of the masses of endothelial cells or the irregularly dilated luminal spaces. Plain computed tomography (CT) of the brain revealed a high density area indicating a hemorrhage (arrow head, Fig. 4A), and contrast CT showed an enhanced area indicating a hemangioma (arrow head, 4B) at 3 months of age. In total, two hemangiomas were found. The hemorrhage was absorbed by 3 months. No neurological signs due to the hemorrhage or the hemangiomas were noted. At one year of age, the number and size of the cherry-red papules were still increasing (Fig. 5).
Comment No giant hemangioma like that found in the present case has been reported in CMH. One
287
Fig. 2. A chest Roentgenogram at birth. The left lung was compressed by the tumor.
case with a similar giant hemangioma on the axilla was reported without the association of CMH (4). In the present case, the giant hemangioma histologically consisted of masses of endothelial cells embedded within fibrous tissue, indicating that the tumor belonged to CMH. The systolic bruit and the echographic findings of the tumor indicated an arteriovenous shunt; however, angiography was not performed. This giant hemangioma, associated with thrombocytopenia and multiple hemangiomas in the skin, buccal mucosa, and brain, indicated that the present case was a type of diffuse neonatal hemangiomatosis. On the other hand, the histological findings, including the irregularly dilated luminal spaces lined with endothelial cells, the masses of endothelial cells with numerous small lumina, and the thrombi, may indicate an association with intravascular papillary endothelial hyperplasia (IPEH). However, no structures of the
288
Sayama et al
Fig. 3A. Photomicrograph of the axillary tumor. Masses of endothelial cells are embedded in fibrous tissues. Some lumina are irregularly dilated and are lined by a single layer of endothelial cells. (Hematoxylin-eosin, original magnification x20).
vascular wall were identified. The association with IPEH and the considerable amount of fibrous tissue may be attributed to repeated hemorrhage and thrombosis within the tumor,
which may also have contributed to the growth of the tumor. Platelet and fibrinogen consumption ceased after tumor resection, suggesting that the consumption took place within the
Diffuse Neonatal Hemangiomatosis
289
Fig. 3B. Photomicrograph of the axillary tumor. Higher magnification of the mass of endothelial cells. (original magnification x200).
tumor. The rate of involvement of the brain has been reported to be 52% in 25 cases of diffuse neonatal hemangiomatosis; it is often asso-
dated with seizures or hydrocephalus (5), and the mortality is high, more than 50% (5, 6). In the pr~sent case, the largest hemangioma could be excised, and the hemangiomas in the brain
Sayama et al
290
Fig. 4. Computed tomography (CT) of the brain at 3 months of age. A: plain CT. The high density area (arrow head) indicates a hemorrhage. B: contrast CT. The enhanced area (arrow head) indicates a hemangioma.
have not been large or frequent enough to induce neurological signs. These factors may contribute to the prognosis.
References 1) Holden KR, Alexander F: Diffuse neonatal hemangiomatosis, Pediatrics, 46: 411-421, 1970. 2) Artherton DJ, Rook A: Naevi and other developmental defects, in Rook A, Wilkinson DS, Ebling FJG, et al (eds): Textbook of Dermatology, Blackwell, London, 1986, pp 207-208. 3) Stem JK, Wolf JE Jr, Jarratt M: Benign neonatal hemangiomatosis,] Am Acad Dermatol; 4: 442-445, 1981. 4) Lofland GK, Filston HC: Giant cutaneous heman-
Fig. 5. Numerous cherry-red papules on the trunk at one year of age. gioma associated with axillary arteriovenous fistula causing congestive heart failure in the newborn infant,] PediatrSurg, 22: 458-460, 1987. 5) Golitz LE, Rudikoff'], O'Meara OP: Diffuse neonatal hemangiomatosis, PediatrDermatol, 3: 145-152, 1986. 6) Kennedy eTC, Fleming P, Duncan A: Eruptive cutaneous and visceral neonatal baemangiomatosis, Br] Dermatol, 123 (Suppl): 83-85, 1990.
