Inclusion Criteria

Inclusion criteria

1. Histologically confirmed diagnosis of malignant PEComa (included epithelioid AML) of
primary disease or of metastatic lesion from archival tissue if it has been obtained
within 12 months prior to enrollment in this study. This histological diagnosis
includes immunochemistry as follows:

- Immunohistochemically positive expression of a melanocytic marker (HMB45, MelanA
or microphtalmia transcription factor) AND of a smooth muscle marker (smooth
muscle actin, pan-muscle actin, h-caldesmon or calponin) is mandatory on primary
or metastatic tumor biopsy.

- Note: According to Folpe (2002), criteria for malignancy in non-AML PEComas are:

- tumor size of more than 5 cm,

- infiltrative growth pattern,

- high nuclear grade,

- mitotic activity of more than 1/50 high power field (HPF),

- necrosis,

- vascular invasion

- Thus, to be included in the trial, the patient should have tumor presenting with
2 or more criteria for malignancy associated with aggressive clinical behavior.

2. If the primary diagnosis was performed more than 12 months before enrollment, the
histology of a primary/metastatic lesion should be reconfirmed with a fresh biopsy.

3. Availability of a representative tumor specimen, either archival or fresh tumor
tissue for PI3K pathway analysis.

4. Unresectable/advanced and/or metastatic and documented progressive measurable disease
as defined by RECIST 1.1 criteria. Prior to study entry, the progression of the
disease should be confirmed by at least 2 sequential CT scans available for
documentation (will be collected and hold).

5. Presence of measurable disease according to RECIST 1.1. Note: Lesions in previously
irradiated areas can only be considered measurable if they have clearly progressed
since the radiotherapy.

6. Treated with 1 or 2 prior lines of treatment Exclusion criteria

1. Disease exclusions:

1. Lymphangioleiomyomatosis (LAM) exclusively

2. Active uncontrolled or symptomatic CNS metastases Note: A patient with controlled and
asymptomatic CNS metastases may participate in this trial. As such, the patient must
have completed any prior treatment for CNS metastases > 28 days (including
radiotherapy and/or surgery) prior to start of treatment in this study and should not
be receiving chronic corticosteroid therapy for the CNS metastases.

3. Concurrent malignancy or malignancy in the last 3 years prior to start the study
treatment (with the exception of adequately treated cervical carcinoma in situ or
nonmelanoma skin cancer).