Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences

Abstract

Background Kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE) are rare and aggressive vascular disorders. The aim of this study was to examine the clinical features and prognosis of KLA and KHE involving the thorax. Methods The clinical features, imaging and pathological findings, treatments and outcome were retrospectively reviewed for 6 patients with KLA and 7 patients with KHE involving the thorax. Results The mean ages at the time of the presentation of signs/symptoms were 26.7 months and 4.1 months for KLA and KHE, respectively. Respiratory symptoms, pericardial and pleural effusion, thrombocytopenia and coagulopathy were common in both KLA and KHE. Diffuse lesions involving the lung and extrathoracic sites were observed in KLA but not in KHE. Histopathologically, all lesions had spindled tumor cells, which were immunoreactive for CD31 and D2–40. In KLA, the spindle cells were distributed in sparse and poorly marginated clusters, whereas the spindle cells formed more defined and confluent vascularized nodules in KHE. Unlike the refractory behavior of KLA, the majority of patients with KHE responded to medical treatments with regression of the lesion and normalization of the hematologic parameters. Conclusions The presenting and histological characteristics of KLA can overlap with those of KHE. The presence of diffuse vascular lesions in the mediastinum and lung with refractory thrombocytopenia and coagulopathy should suggest the diagnosis of KLA. Given the rarity and high morbidity and mortality of these disorders, the diagnostic process and therapeutic approach should include a multidisciplinary team review and consensus. Electronic supplementary material The online version of this article (10.1186/s13023-019-1147-9) contains supplementary material, which is available to authorized users.

Kaposiform hemangioendothelioma: current knowledge and future perspectives

“…Interestingly, thrombocytopenia and coagulopathy in KLA have overlapping features with KMP in KHE. The thrombocytopenia in some KLAs is extremely severe, similar to that observed in KMP [98,99]. Histologically, KLAs are composed of malformed lymphatic channels with dispersed and poorly marginated clusters or sheets of spindled lymphatic ECs [96,98].…”

“…The thrombocytopenia in some KLAs is extremely severe, similar to that observed in KMP [98,99]. Histologically, KLAs are composed of malformed lymphatic channels with dispersed and poorly marginated clusters or sheets of spindled lymphatic ECs [96,98]. In vitro, KHE cells can support vascular network formation, whereas KLA cells appear inert in this capacity [35].…”

Curative effect of surgical resection in the treatment of Kaposiform hemangioendothelioma

“…Several vascular lesions such as Kaposiform lymphangiomatosis (KLA), infantile haemangioma (IH) resemble KHE clinically 11 , and may be localized in the mediastinum, lung, and thorax, etc. 12 . Thus, investigate the occurring rule of predilection sites might be beneficial for the KHE diagnosis.…”

How we approach coagulopathy with vascular anomalies.

“…Kaposiform lymphangiomatosis (KLA) is a very rare lymphatic disorder that usually presents with multifocal or diffuse involvement of the mediastinum, lungs, bones and abdomen. These patients can have very severe thrombocytopenia with coagulopathy similar to KMP [18][19][20] . They do not typically respond to corticosteroids.…”