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Kaposiform lymphangiomatosis is an uncommon generalized lymphatic anomaly with distinctive clinical, radiologic, histopathologic, and molecular findings. Herein, we document the pathology in 43 patients evaluated by the Boston Children's Hospital Vascular Anomalies Center from 1999 to 2020. The most frequent presentations were respiratory difficulty, hemostatic abnormalities, and a soft tissue mass. Imaging commonly revealed involvement of some combination of mediastinal, pulmonary, pleural, and pericardial compartments and most often included spleen and skeleton. Histopathology was characterized by dilated, redundant, and abnormally configured lymphatic channels typically accompanied by dispersed clusters of variably canalized, and often hemosiderotic, spindled lymphatic endothelial cells that were immunopositive for D2-40, PROX1, and CD31. An activating lesional NRAS variant was documented in 9 of 10 patients. The clinical course was typically aggressive, marked by hemorrhage, thrombocytopenia, diminished fibrinogen levels, and a mortality rate of 21%.

Objectives Hepatic hemangiomas are often found in association with multiple cutaneous infantile hemangiomas. Screening abdominal ultrasonography has been recommended for patients with five or more cutaneous lesions. We sought to determine whether hemangiomas found through screening had improved clinical outcomes. Methods Patients entered into our hepatic hemangioma registry between 1995 and 2012 were reviewed. Results Seventy-two patients with multiple cutaneous and hepatic hemangiomas were identified; 43 (60%) were detected through screening. The median age at diagnosis was 41 days for screened patients and 53 days for those not screened. Screening detected 40 (93%) multifocal and 3 (7%) diffuse hemangiomas, compared to 18 (62%) and 11 (38%), respectively, in the nonscreened group. Patients identified by screening had lower incidences of congestive heart failure and hypothyroidism and were less likely to receive treatment for their hemangiomas. The mortality rate in the children not screened was 28% (n = 8). None of the patients found by screening died (p

In the past pyogenic granuloma (PG) in infancy has been easily confused clinically and histopathologically with infantile hemangioma (IH). In 2000 North and colleagues discovered that IH is immunopositive for GLUT-1, a glucose transporter which is also expressed in placental capillaries (1). GLUT-1 staining is negative in pyogenic granuloma. We report two newborns with congenital disseminated PG who were otherwise healthy. In both of these cases, negative GLUT-1 staining supported the proper diagnosis.

We describe six patients with an uncommon variant of infantile hemangioma that we have termed reticular, occurring in the extremity, which were associated with intractable ulceration, anogenito-urinary-sacral anomalies, and sometimes cardiac overload. The extreme end of the spectrum is exemplified by a male neonate who presented with a stained, enlarged, pulsatile lower extremity, and cardiac failure. He also had hepatic hemangiomas and ambiguous genitalia. Progressive soft tissue necrosis and bony destruction necessitated amputation. The histopathologic features differed from those of typical infantile hemangioma: infiltrative (not lobular) and involving fascia, muscle, and bone. The mid-spectrum is illustrated by five females with reticular infantile hemangioma of the lower limb, buttock, and perineum. Four of these infants had a ventral-caudal anomaly, including omphalocele, recto-vaginal fistula, solitary/duplex kidney, imperforate anus, and tethered cord; one infant also had hepatic hemangiomas. Deep ulcerations healed following corticosteroid therapy; one patient required skin graft for closure of a thigh wound. The minor end of the spectrum is exemplified a patchy lesion in the distal limb. The reticular variant of infantile hemangioma can be confused with other vascular anomalies in the limb, such as capillary malformation, cutis marmorata telangiectasia congenita, diffuse arteriovenous malformation (Parkes Weber syndrome) and capillary-lymphatico-venous malformation (Klippel-Trenaunay syndrome). The macular network-like appearance of the tumor and coexisting ventral-caudal structural anomalies is analogous to the association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities association in the craniofacial region.

We analyzed the clinical and histopathologic characteristics of verrucous hemangioma, compared these findings to hyperkeratotic mimickers such as capillary-lymphatic malformation or capillary-venous malformation and angiokeratoma circumscriptum, and reconsidered whether the term verrucous hemangioma is appropriate in the current nosology of vascular anomalies. Fourteen similar-appearing localized hyperkeratotic vascular lesions were identified by one surgeon as either angiokeratoma, angiokeratoma circumscriptum, capillary-venous malformation, capillary-lymphatic malformation, or verrucous hemangioma. All lesions were located on an extremity, except for one lesion on the trunk, and were single or grouped and 2.6 to 8 cm in diameter. All were raised, red-to-purple, variably keratotic with irregular borders, and several manifested intermittent bleeding and oozing. Excision was performed at 4 months to 16 years of age. After histologic review, three lesions were designated as combined vascular malformations composed of capillaries, lymphatics, and veins, and none was designated as angiokeratoma. Eleven of 14 specimens met the histologic criteria for verrucous hemangioma: a hyperkeratotic epidermis with small, thick-walled, blood-filled vessels with multilamellated basement membrane involving the entire dermis as well as the subcutis. Immunostaining showed focal GLUT1 endothelial positivity (7/11) and low-level MIB-1 reactivity (8/11). Verrucous hemangioma has the accepted clinical features of vascular malformation, specifically presence at birth and proportionate growth. Microscopic features, such as thick vascular walls, multilamellated basement membrane, relatively uniform channel size, and GLUT1 immunopositivity are reminiscent of infantile hemangioma, particularly in its involutive phase. No firm conclusion seems possible as to whether verrucous hemangioma is a malformation or an indolent tumor, but clinical evidence favors the former category.