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Primary diffuse leptomeningeal atypical teratoid rhabdoid tumor

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病史

Five year old boy with progressive headache since one month. Vomiting. Weight Loss.

 


檢查結果

Diffuse homogeneous abnormal spinal cord uptake (SUVmax 2.5). No other suspicious lesion.

 

DDx

Primary diffuse leptomeningeal gliomatosis

Lymphoma

CNS infection

Leptomeningeal carcinomatosis


診斷

Primary diffuse leptomeningeal atypical teratoid rhabdoid tumor


討論

Atypical rhabdoid/teratoid tumor (AT/RT) is a highly malignant tumor accounting for 10% of central nervous system tumors in infants. This neoplasia is characterized histologically bynests or sheets of rhabdoid tumor cells with varying proportions of primitive neuroectodermal tumor (PNET) cells, mesenchymal spindle-shaped tumor cells, and/or epithelial-type tumor cells.

 

This tumor has aggressive growth with high potential for dissemination and poor prognosis.

In children, the most common primary site is the cerebellum. Leptomeningeal spread is a well known aggressive clinical course of AT/RT and is reported to occur in one third of paediatric cases at presentation. However, a focal parenchymal lesion is usually present.

At the present, there are only four cases of primary diffuse leptomeningeal atypical teratoid/rhabdoid tumor reported in the literature.

There was no other primary site to support the leptomeningeal carcinomatosis hypothesis on the FDG PET/CT exam. Furthermore no focal cerebral lesion was present. Differential diagnosis included CNS infection/inflammation and diffuse malignancy of unknown origin.


參考資料

Livermore et al : Primary diffuse leptomeningeal atypical teratoid/rhabdoid tumour in an adolescent. Clin Neurol Neurosurg. 2013 Oct;115(10):2170-3

El-Nabbout et al : Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor: report of the first case. J Neurooncol. 2010 Jul;98(3):431-4

Gauvain et al : Rapidly progressive primary leptomeningeal atypical teratoid/rhabdoid tumor: a report of 2 cases. J Child Neurol. 2012 Dec;27(12):1597-601

Meyers et al : Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR Am J Neuroradiol. 2006 May;27(5):962-71



作者
Dr Marc-André Levasseur , Dr Sophie Turpin , Dr Raymond Lambert
机构
Université Montréal, Université Sherbrooke
频道
Pediatric PET/CT cases
Primary diffuse leptomeningeal atypical teratoid rhabdoid tumor  MIP body

MIP body

Primary diffuse leptomeningeal atypical teratoid rhabdoid tumor  Fusion body

Fusion body

Primary diffuse leptomeningeal atypical teratoid rhabdoid tumor  Body-Low Dose CT

Body-Low Dose CT

Primary diffuse leptomeningeal atypical teratoid rhabdoid tumor  CTAC body

CTAC body

Primary diffuse leptomeningeal atypical teratoid rhabdoid tumor  MIP head

MIP head

Primary diffuse leptomeningeal atypical teratoid rhabdoid tumor  Fusion head

Fusion head

Primary diffuse leptomeningeal atypical teratoid rhabdoid tumor  Head-Low Dose CT

Head-Low Dose CT

Primary diffuse leptomeningeal atypical teratoid rhabdoid tumor  CTAC head

CTAC head

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