Desmoplastic Small Round Cell Tumor of the Pleura Successfully Treated with a Lower Dose of Pazopanib

Abstract

Desmoplastic small round cell tumor (DSRCT) is an aggressive mesenchymal tumor which primarily affects the abdomen. Even a multimodal approach rarely achieves durable remission and the optimal therapy for extended disease is unknown. We herein describe a rare case of DSRCT arising from the pleura in a 32-year-old man. Initial therapy, which included chemotherapy, surgery and radiotherapy, achieved a partial response for only two months. Although salvage chemotherapies had no effect, pazopanib treatment shrank the tumors and was well-tolerated on an outpatient basis. From the viewpoint of quality of life, pazopanib may therefore be a good therapeutic option for this aggressive disease.

Clinical Activity of Pazopanib in Patients with Advanced Desmoplastic Small Round Cell Tumor

“…Clinical benefit from pazopanib was observed in 62% of our patient population (CR in 1 patient, PR in 1 patient, SD in 16 patients). In general, this clinical benefit was transient, with 61% of these patients remaining progression-free less than 6 months, an interval consistent with previous reports [31]. Although the majority of these responses were short-lived, clinical benefit at 12 weeks was associated with increased OS as assessed using a landmark analysis.…”

Desmoplastic small round cell tumors of the pleura: a review of the clinical literature

“…Fifteen cases included in fourteen articles were retrieved [ 6 – 19 ]. Cases with poor clinical data were excluded, as well as those in which the pleural origin was not clearly stated or demonstrated, and those reported in more than one article [ 6 , 8 , 20 – 23 ].…”

“… 34 Free of disease Quarssani et al, 2011 [ 14 ] Radiation, chemotherapy 53 Alive with disease Benbrahim et al, 2012 [ 15 ] Chemotherapy 5 Alive with disease Jian et al [ 16 ] Chemotherapy 22 Dead. Cao et al [ 17 ] Surgery 32 Free of disease Won et al, 2015 [ 18 ] Chemotherapy 16 Dead Ikeue et al [ 19 ] Chemotherapy, surgery, radiation 6 Alive with disease …”

“…In cases of advanced tumors contrast enhancement is often heterogeneous, showing low attenuation areas that correspond to myxoid alterations, hemorrhage, necrosis or cystic degeneration; these findings and the pleural effusion can be bilateral in such cases [ 6 , 17 ]. CT scans for the detection of distant metastases have been performed in most of the cases reviewed; nevertheless, distant metastases are rare, but extension along the crura of the diaphragm to the retroperitoneum has been reported [ 19 ]. Also, local invasion of osseous anatomical structures (ribs or vertebrae) has been found, generally evaluated also with MRI or Tc-99m MDP bone scans [ 9 , 13 ].…”

Tumor Desmoplásico de Pequenas Células Redondas: Relato de Caso

“…O erlotinibe, um inibidor multialvo da tirosina quinase, levou à resposta parcial em um relato de caso 19 . A atividade contra a doença foi relatada com uso de pazopanibe 20,21 e eribulin 22 . Relato recente demonstrou resultados negativos com imatinibe 23 bevacizumabe também foi relatada [24][25][26] .…”