We present a case of congenital cutaneous multifocal kaposiform hemangioendothelioma (KHE). [ncbi.nlm.nih.gov]

• Splenectomy

  Because of the rapid onset of thrombocytopenia and anemia, the patient required urgent splenectomy with subsequent resolution of the blood dyscrasias. [ncbi.nlm.nih.gov]

  A splenectomy was performed, and the patient was discharged without any additional complications. [karger.com]

• Soft Tissue Mass

  It most commonly presents as a superficial or deep soft tissue mass with associated cutaneous lesions. [ncbi.nlm.nih.gov]

  Lymphangiomatosis consisted of diffusely infiltrating lymphangioma of soft tissue (three cases) and in two by the additional presence of bone lesions. [journals.lww.com]

• Cushingoid

  Steroids have the advantage that they can be given by mouth but do have side effects which include delayed growth, cushingoid appearance, high blood pressure and increased risk of infection, to name a few. [novanews.org]

• Chills

  Interferon needs to be given as an injection once a day and is associated with side effects such as flu-like symptoms (fever, chills) and neurological complications. [novanews.org]

• Episodic Pain

  In the subsequent years, she experienced episodic pain at the same site, but no specific therapy was given. [nature.com]

• Sore Throat

  A 2-year-old male child referred to us with history of sore throat, dyspnoea and right-sided neck swelling off and on, since birth, was clinicoradiologically diagnosed with recurrent tonsillitis, including right sided peritonsillar abscess, for which [ncbi.nlm.nih.gov]

• Abdominal Tenderness

  Abdominal tenderness in the left upper quadrant was noteworthy. A computed tomography (CT) scan revealed a 16 × 11 × 24 cm splenic mass with signs of splenic rupture. [karger.com]

• Odynophagia

  There were no associated symptoms of deviation of the tongue, change in taste, numbness, dysphagia, odynophagia, dysphonia, or shortness of breath. The patient did report a lifelong history of slight articulation problems with the letters T and S. [current-oncology.com]

• Jaw Pain

  Its main complications are constipation and jaw pain but the drug can have some other neurotoxicity. It is given once a week until an improvement is seen and then the interval of administration is increased. [novanews.org]

• Dysarthria

  Postoperatively, the patient has recovered completely, with minimal residual post-operative dysarthria, which has been improving with time. [current-oncology.com]

Initial workup demonstrated large pleural and pericardial effusions. The patient had no cutaneous abnormality on physical examination, and his initial chest CT (computed tomography) was nondiagnostic. [ncbi.nlm.nih.gov]

Progress logically from the histologic pattern, through the appropriate workup, around the pitfalls, to the best diagnosis. [books.google.com]

See Workup for more detail. Management No single pharmacologic therapy has been proved most effective in patients with KMS. [emedicine.medscape.com]

Two patients developed grade 2 oral mucositis during treatment. CONCLUSION: Sirolimus as first-line therapy shows great promise in the treatment of kaposiform hemangioendothelioma and tufted angioma. © 2018 Wiley Periodicals, Inc. [ncbi.nlm.nih.gov]

Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon. [scholars.northwestern.edu]

Intestinal type KHE showed better prognosis if the lesion was limited in the gastrointestinal tract and coagulopathy was not accompanied. Since the number of reported cases is small, further study for treatment options and prognosis need to be done. [ncbi.nlm.nih.gov]

Prognosis - Kaposiform Hemangioendothelioma (KHE) Treatment - Kaposiform Hemangioendothelioma (KHE) Resources - Kaposiform Hemangioendothelioma (KHE) [checkorphan.org]

The tumor's etiology remains unknown and there are no specific treatments. In this study, we performed exome sequencing using DNA from a Kaposiform hemangioendothelioma patient, and found putative candidates for the responsible mutations. [ncbi.nlm.nih.gov]

The etiology of kaposiform hemangioendothelioma remains unknown. It is not associated with HHV8 infection or Kaposi sarcoma. Tumors often involve soft tissue of extremities as ill-defined, violaceous lesions and may encase normal structures. [path.upmc.edu]

They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]

(Etiology) Even though Kaposiform Hemangioendothelioma occurrence is congenital, there is no genetic factor involved in its formation. [dovemed.com]

Expression of D2-40 was identified in lymphatic endothelium of normal tissues and in vascular tumors, with 3 of 3 Dabska tumors being positive for D2-40. [11] Etiology No causes have been linked to this type of tumor. [emedicine.medscape.com]

Epidemiology [ edit ] It is so rare that only 0.01 percent of the cancer population has it and it affects about 1 person in every 1,000,000 worldwide. [4] Around 20 cases are diagnosed in America every year – the cause is unknown. [5] It is unresponsive [en.wikipedia.org]

Epidemiology Frequency The incidence of DT is unknown. Only approximately 30 patients have been described worldwide. Race No ethnic or racial predilection is evident. Sex No predilection is yet evident. [emedicine.medscape.com]

Presumed to be that of platelet trapping 6 Pathophysiology How platelets become trapped not clear Physical entrapment Adhesion to abnormally proliferating endothelium within the hemangioma (Gilon, 1959) 7 Pathophysiology The PLT are consumed in the process [docplayer.net]

Pathophysiology Controversy remains about the DT, particularly whether it is a unique disorder or a heterogeneous group of neoplasms. DT may be viewed as a distinct intralymphatic neoplasia and renamed papillary intralymphatic angioendothelioma. [emedicine.medscape.com]

Prevention - Kaposiform Hemangioendothelioma (KHE) Cannot be prevented. Diagnosis - Kaposiform Hemangioendothelioma (KHE) If a patient is suspected to have this condition, blood tests will be performed, an MRI and a biopsy can also be performed. [checkorphan.org]

To prevent development of amblyopia, this mass was surgically excised on the sixth day of life. [ncbi.nlm.nih.gov]

Ideally, thorough treatment will prevent problems later in life, including tumor recurrence. Some tumors shrink and even become invisible with therapy. KHE treatment takes an interdisciplinary, team-based approach. [cmtcovm-us.org]