Pediatric Annals

A 6-month-old Boy With an Enlarging Bruise on His Back

Erin Mathes, MD; Marion Koerper, MD; Ilona Frieden, MD

Abstract

In this case, an incisional biopsy was performed to exclude the possibility of another soft-tissue neoplasm, such as a sarcoma. This biopsy demonstrated a vascular neoplasm with areas of cannonball like-tufts admixed with spindled cells, demonstrating features of both tufted TA and KHE.

The patient was started on prednisolone at a dose of 2 mg/kg/day and aspirin to treat his tumor, and trimethoprim-sulfamethoxasole for infectious prophylaxis. He was otherwise stable, and because his platelet count normalized, he was discharged from the hospital. One week later, the mass had enlarged, with increasing ecchymosis and serosanguinous drainage from the biopsy site. He was therefore readmitted to the hospital for further treatment.

On readmission, the mass was 9 by 9 cm, with an expanded area of overlying ecchymosis. His laboratory studies were remarkable for platelets 40 ? 109/L (nl 140-450), D-dimers greater than 10,000 ng/mL (nl < 1000), and fibrinogen less than 35 mg/dL (nl 175-433). He was given pulse-dosed solumedrol (30 mg/kg/ day) for three days, started on vincristine, and transfused platelets and packed red blood cells. Interventional radiology was consulted and performed an angiogram and embolization of branches of the right subclavian artery that fed the tumor. His CBC and fibrinogen were normal on discharge, and he has been stable since.

1. Enjolras O, Wassef M, Mazoyer E, et al. Infants with Kasabach-Menitt syndrome do not have "true" hemangiomas. J Pediatr. 1997;130(4):631-640.

2. North PE, Waner M, Mìzeracki A, et al. A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol. 2001;137(5):559-570.

3. Muzaffar AR, Friedrich JB, Lu KK, Hanel DP. Infantile fibrosarcoma of the hand associated with coagulopathy. Plast Recensir Surg. 2006;117(5):81e-86e.

4. Jones EW, Orkin M. Tufted angioma (angioblastoma). A benign progressive angioma, not to be confused with Kaposi's sarcoma or lowgrade angiosarcoma. J Am Acad Dermatol. 1989;20(2Pt l):214-225.

5. Zukerberg LR, Nickoloff BJ, Weiss SW. Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol 1993; 17(4):32 1-328.

6. Bolongia J, Jorizzo J, Rapini R. Dermatology. London, England: Mosby; 2003: 1826-1828.

7. Yin-Christian K, McCalmont TH, Frieden IJ. Kaposiform hemangioendothelioma. An aggressive, locally invasive vascular tumor that can mimic hemangioma of infancy. Arch Dermatol. 1997;133(12): 1573-1578.

8. Herrón MD, Coffin CM, Vanderhooft SL. Tufted angiomas: variability of the clinical morphology. Pediatr Dermatol. 2002; 19(5): 394401.

9. Gruman A, Liang MG, Mulliken JB, et al. Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon. J Am Acad Dermatol. 2005;52(4):616-22.

10. Haisley-Royster C, Enjolras O, Frieden D, et al. Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. 2002;24(6):459-462.

11. Munn SE, Jackson JE, Jones RR. Tufted haemangioma responding to high-dose systemic steroids: a case report and review of the literature. Clin Exp Dermatol. 1994;19(6):511-514.

12. Eichenfield LF, Frieden IJ, Esterly NB. Textbook of Neonatal Dermatology. Philadelphia, PA: WB Saunders; 2001:346-348.

13. Enjolras O, Mulliken JB, Wassef M, et al. Residual lesions after Kasabach-Merritt phenomenon in 41 patients. JAm Acad Dermatol. 2000;42(2Ptl):225-35.

14. Mathes EFD, Haggstrom AN, Dowd C, Hoffman W, Frieden II. Clinical characteristics and management of vascular anomalies: findings of a multidisciplinary vascular anomalies clinic. Arch Dermatol. 2004;140(8):979-983.

TABLE.

Differential Diagnosis of Infantile Hemangioma,Tufted Angioma, and Kaposiform Hemangioendothelioma…

A 6-month-old boy presented for evaluation of an enlarging lump with bruising on his posterior neck. The mass was first noticed 1 month earlier when it was quarter-sized with overlying bruising. He was initially treated with antibiotics. The mass shrank, then enlarged, growing to the size of an orange. A complete blood count ordered by his pediatrician showed thrombocytopenia and anemia. Other than mild upper respiratory infection symptoms, he was well, with no history of fever, trauma, bleeding, or bruising elsewhere on his body. Birth, medical, developmental, and family history were negative for abnormalities.

Physical examination revealed a 4 by 8 cm firm, nontender mass with overlying ecchymosis and purpura on his upper back and lower posterior neck just lateral to midline (Figure). The remainder of the physical examination was normal.

Laboratory studies included hemoglobin of 9.9 g/dL (nl 11-13.5), platelets 80 x 109 (nl 140-450), and fibrinogen 134 mg/dL (nl 175-433). A magnetic resonance imaging scan demonstrated a large infiltrating mass in the lower posterior neck with cervical and right axillary lymphadenopathy.

DIAGNOSIS

Kasabach-Merritt phenomenon due to tufted angioma, with some histologie features of kaposiform hemangioendothelioma

DISCUSSION

The leading diagnostic consideration was a vascular tumor such as a tufted angioma (TA) or kaposiform hemangioendothelioma (KHE), with accompanying Kasabach-Merritt phenomenon (KMP). KMP is consumptive coagulopathy and thrombocytopenia caused by platelet trapping within a vascular tumor. Diagnosis is based on thrombocytopenia, low fibrinogen, and coagulopathy of varying degrees in the setting of an enlarging vascular tumor.

For many decades, KMP was believed to be a complication of infantile hemangioma, but it is now recognized that it does not occur in infantile hemangioma, but rather is associated almost exclusively with TA and KHE.1 Infantile hemangiomas recently have been discovered to have a set of unique immunohistochemical markers, including glucose transporter iosoform 1 (GLUTl), which help differentiate them from other vascular tumors (including TA and KHE) and malformations which do not have GLUTl staining.2 Biopsy of the tumor sometimes is necessary to differentiate it from other tumors, as some, such as infantile fibrosarcoma, can initially present with a coagulopathy.3

TA and KHE are rare vascular tumors that have been described in the last 25 years.4'5 TA, which also has been called "angioblastoma of Nakagawa," is characterized by round aggregates of tightly packed capillaries (so-called "cannonballs") without prominent spindle cells. KHE, first described in 1993 by Zuckerberg et al.,5 is characterized by ill-defined coalescing nodules of plump spindle cells that form slit-like lumina. The features of TA and KHE can be distinctive, but some cases, like ours, show both histologie patterns, suggesting that they may part of a histologie spectrum, rather than distinct tumors. Some authors believe that TA represents a milder, more superficial form of KHE.1'6 Neither tumor can result in distant metastatic spread, but KHE has been reported to spread to local lymph nodes, and can cause death via direct tumor infiltration of vital structures.7

Most tufted angiomas are either present at birth or acquired in early childhood, with 50% of cases presenting by age 5.4 They can present as red-purple, ill-defined patches that can enlarge into indurated nodules and plaques, most often on the upper torso, shoulders, and neck, with a lateral growth phase that can lasts months to years.8 In patients who develop KMP, the tumor can grow rapidly, developing a dusky color with superimposed and surrounding purpura and ecchymosis. Some lesions are painful, especially during periods of platelet trapping. Increased overlying hair growth may be present. Spontaneous involution is seen occasionally, but much less commonly than in infantile hemangioma. KHE also can occur in the absence of an associated coagulopathy,9 but the relative lack of such reports compared to TA suggests that it more often has an associated coagulopathy.

No single therapy or combination of therapies is uniformly effective in KMP, but high-dose corticosteroids and vincristine are often first-line therapies.10'11 Other treatment modalities include wide local excision (which is ideal, if the lesion is small enough to excise), arterial embolization of feeder vessels, aspirin and ticlopidine, interferon-alfa, radiation therapy, and combinations of several chemotherapeutic agents.10 While platelet transfusions may be necessary in certain cases, there have been reports of platelet transfusions causing growth of the tumor and worsening of the coagulopathy, perhaps via release of angiogenesis factors such as platelet-derived growth factor.4 Based on these reports, platelets should be avoided if possible unless active bleeding is occurring or a surgical procedure is planned. Even once the KMP has resolved, residual tumor burden with pain, fibrosis, and other sequelae are relatively common.13 Such patients are best managed in a multidisciplinary setting, ideally by a vascular anomalies team.14

The differential diagnosis of a rapidly growing soft-tissue tumor in an infant includes infantile hemangioma (the most common tumor of infancy), Kaposi sarcoma (rare in infancy and childhood), vascular malformations, fibrosarcoma, rhabdomyosarcoma, Iipoblastoma, and fibroblastoma, among others. Both the presence of a fully formed tumor at the time of birth and onset of growth after age 3 to 4 months can be important clues that differentiate the above tumors from an infantile hemanigoma (Table).8

Table

TABLE.Differential Diagnosis of Infantile Hemangioma,Tufted Angioma, and Kaposiform Hemangioendothelioma

TABLE.

Differential Diagnosis of Infantile Hemangioma,Tufted Angioma, and Kaposiform Hemangioendothelioma

In this case, an incisional biopsy was performed to exclude the possibility of another soft-tissue neoplasm, such as a sarcoma. This biopsy demonstrated a vascular neoplasm with areas of cannonball like-tufts admixed with spindled cells, demonstrating features of both tufted TA and KHE.

The patient was started on prednisolone at a dose of 2 mg/kg/day and aspirin to treat his tumor, and trimethoprim-sulfamethoxasole for infectious prophylaxis. He was otherwise stable, and because his platelet count normalized, he was discharged from the hospital. One week later, the mass had enlarged, with increasing ecchymosis and serosanguinous drainage from the biopsy site. He was therefore readmitted to the hospital for further treatment.

On readmission, the mass was 9 by 9 cm, with an expanded area of overlying ecchymosis. His laboratory studies were remarkable for platelets 40 ? 109/L (nl 140-450), D-dimers greater than 10,000 ng/mL (nl < 1000), and fibrinogen less than 35 mg/dL (nl 175-433). He was given pulse-dosed solumedrol (30 mg/kg/ day) for three days, started on vincristine, and transfused platelets and packed red blood cells. Interventional radiology was consulted and performed an angiogram and embolization of branches of the right subclavian artery that fed the tumor. His CBC and fibrinogen were normal on discharge, and he has been stable since.

REFERENCES

1. Enjolras O, Wassef M, Mazoyer E, et al. Infants with Kasabach-Menitt syndrome do not have "true" hemangiomas. J Pediatr. 1997;130(4):631-640.

2. North PE, Waner M, Mìzeracki A, et al. A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol. 2001;137(5):559-570.

3. Muzaffar AR, Friedrich JB, Lu KK, Hanel DP. Infantile fibrosarcoma of the hand associated with coagulopathy. Plast Recensir Surg. 2006;117(5):81e-86e.

4. Jones EW, Orkin M. Tufted angioma (angioblastoma). A benign progressive angioma, not to be confused with Kaposi's sarcoma or lowgrade angiosarcoma. J Am Acad Dermatol. 1989;20(2Pt l):214-225.

5. Zukerberg LR, Nickoloff BJ, Weiss SW. Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol 1993; 17(4):32 1-328.

6. Bolongia J, Jorizzo J, Rapini R. Dermatology. London, England: Mosby; 2003: 1826-1828.

7. Yin-Christian K, McCalmont TH, Frieden IJ. Kaposiform hemangioendothelioma. An aggressive, locally invasive vascular tumor that can mimic hemangioma of infancy. Arch Dermatol. 1997;133(12): 1573-1578.

8. Herrón MD, Coffin CM, Vanderhooft SL. Tufted angiomas: variability of the clinical morphology. Pediatr Dermatol. 2002; 19(5): 394401.

9. Gruman A, Liang MG, Mulliken JB, et al. Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon. J Am Acad Dermatol. 2005;52(4):616-22.

10. Haisley-Royster C, Enjolras O, Frieden D, et al. Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. 2002;24(6):459-462.

11. Munn SE, Jackson JE, Jones RR. Tufted haemangioma responding to high-dose systemic steroids: a case report and review of the literature. Clin Exp Dermatol. 1994;19(6):511-514.

12. Eichenfield LF, Frieden IJ, Esterly NB. Textbook of Neonatal Dermatology. Philadelphia, PA: WB Saunders; 2001:346-348.

13. Enjolras O, Mulliken JB, Wassef M, et al. Residual lesions after Kasabach-Merritt phenomenon in 41 patients. JAm Acad Dermatol. 2000;42(2Ptl):225-35.

14. Mathes EFD, Haggstrom AN, Dowd C, Hoffman W, Frieden II. Clinical characteristics and management of vascular anomalies: findings of a multidisciplinary vascular anomalies clinic. Arch Dermatol. 2004;140(8):979-983.

TABLE.

Differential Diagnosis of Infantile Hemangioma,Tufted Angioma, and Kaposiform Hemangioendothelioma

10.3928/0090-4481-20060601-10

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