Symptoma

Kaposiform Hemangioendothelioma

Congenital Cutaneous Multifocal Kaposiform Hemangioendothelioma

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]

    The American Journal of Surgical Pathology: April 1993 Original Articles: PDF Only Abstract We report the clinical and pathological features of nine distinctive, but relatively unknown, vascular tumors of infancy and childhood presenting as soft tissue [journals.lww.com]

  • Tonsillar Mass

    Wick Case of the Week #401: Low power view CD31 (20x) CD34 SMA Vimentin Images hosted on other servers: "Cannon ball" appearance Slit-like lumens VEGFR3 Tonsillar mass Back to top [pathologyoutlines.com]

    Histopathological sections from the excised tonsillar mass were reviewed and showed a tumor composed of irregular, infiltrating lobules of spindle cells arranged in kaposiform architecture with slit-like, crescentic vessels. [ncbi.nlm.nih.gov]

  • 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]

  • Tachypnea

    This tumor clinically presented as persistent tachypnea and was unresponsive to conventional therapy with vincristine and steroids but responded dramatically to Sirolimus with almost complete regression on follow-up. [ncbi.nlm.nih.gov]

  • Lower Abdominal Pain

    He had experienced lower abdominal pain and fever for several days. Contrast-enhanced CT images revealed a well-enhanced mass involving several large and small cystic parts with thin, enhanced walls at the sigmoid colon. [ncbi.nlm.nih.gov]

  • Vomiting

    We described the case of a 1-month-old female who had abdominal distention and bilious vomiting. She was suspected as intestinal obstruction after diagnostic work up. [ncbi.nlm.nih.gov]

  • Bilious Vomiting

    We described the case of a 1-month-old female who had abdominal distention and bilious vomiting. She was suspected as intestinal obstruction after diagnostic work up. [ncbi.nlm.nih.gov]

  • Palpitations

    […] fluorodeoxyglucose (F-FDG) positron emission tomography (PET)/CT findings in an adult patient with KH associated with lymphangiomatosis involving mesentery and ileum.A 22-year-old female complained of a 9-month history of intermittent melena, weakness, and palpitation [ncbi.nlm.nih.gov]

  • Flushing

    Complications included one patient with flush on face, one patient with needle scars, and one patient with a small area of local tissue necrosis. CONCLUSION: Absolute ethanol therapy presents a safe option in the treatment of KMP. [ncbi.nlm.nih.gov]

  • Musculoskeletal Pain

    Common presenting features included enlarging cutaneous lesion (75%), thrombocytopenia (56%), and musculoskeletal pain or decreased function (23%). Cutaneous KHE favored the extremities, especially overlying joints. [ncbi.nlm.nih.gov]

  • Aggressive Behavior

    Kasabach-Merritt syndrome is a rare type of vascular tumor with aggressive behavior in association with thrombocytopenia and consumptive coagulopathy. A standard guideline has not been established to date. [ncbi.nlm.nih.gov]

    Despite the locally aggressive behavior and the potential for lethal complications due to consumptive coagulopathy in the setting of KMP, metastatic spread of KHE seldom occurs [ 1 ]. [karger.com]

    Thus, the designation of hemangioendothelioma infers to its local aggressive behavior, but no longer for a metastatic potential. [nature.com]

  • Neck Swelling

    Herein, we present an extremely uncommon case of KHE occurring in tonsil of a child, associated with a neck swelling, but unassociated with KMP. [ncbi.nlm.nih.gov]

  • Breast Mass

    We treated a 32-year-old woman with a right breast mass that was diagnosed based on pathologic examination as a KHE. We performed operations several times because of recurrence of KHE and finally performed mastectomy despite the patient's young age. [ncbi.nlm.nih.gov]

  • Unable to Walk

    We report the case of a 10-year-old boy with a KHE of his right distal thigh who was unable to walk without assistance due to fibrotic change and right knee contracture. He had no laboratory evidence of KMP at the time of representation. [ncbi.nlm.nih.gov]

  • Intracranial Hemorrhage

    Unfortunately, a regrowth of KHE with KMS was observed 4 months after the cessation of treatment and the patient thereafter died from an intracranial hemorrhage and Pneumocystis carinii pneumonia, which is a complication related to repetitive radiation [ncbi.nlm.nih.gov]

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]

Twenty-eight patients have ended treatment with VCR; the average length of treatment was 31.2   5.9 weeks. Side effects occurred in 48.6% of patients who received steroids, and in 11.4% of patients who received VCR treatment. [ncbi.nlm.nih.gov]

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]

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]

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

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

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 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]

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]

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]

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]

[…] endothelial cell division have been used to prevent tumor growth. [hemangioma.com]

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