25 yrs old female story:
Episode 1 2008 August: CLINICALLY sarcoma in pelvis minor. Bone pain and walking disability. PATHOLOGY August 2008: casus pro diagnosis (external blocks): "High grade sarcoma, most probably malignant peripheral nerve sheat tumor (MPNST). IH 1: VIMENTIN+, Ki67+ 60%, CD117+ (weak cytoplasmic 20%), S100+ (weak nuclear and cytoplasmic 40%. NEGATIVE: Asmactin/Desmin/PanCK/CD10/CD34/CD3/CD20/EMA/HMB45/Inhib. CHEMOTHERAPY was administered with good reponse (necrosis, below).
Episode 2 2008 September: CLINICALLY tumor in the inguinal area. Sacral 10 x 15 cm tumor with central degeneration was removed. BIOPSY 2008 September: Malignant tumor totally necrotic, histogenesis obscure. IH 2 (Antigeneity lost): Vimentin/LCA/CD99 (-).
Eisode 3 2009 May: CLINICALLY: Pelvic MPNST pT2bNxM1, IV st. Mts in the soft tissue, retroperitoneum, lungs, paraaortic nodes. PATHOLOGY 2009 May: High grade clear cell sarcoma: necrosis 80%. IH 3: Vimentin/S-100: 100%+; Ki-67: ~30%; PanCK+ 10% (focal weak); CD99+ 100% (weak like background staining). NEGATIVE: Cam5.2/AsmActin/Desmin/CD34/EMA/HMB-45/Melan A/CD56/CD57.
MOLECS: LT Ewing and Synovioma specific translocations were not found.
PHOTOS: From the last episode 3: from Apperio virtual slides taken. Some additional photos (more in square format) by Olympus DP10.
Pelvic peripheral nerve sheat tumor