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Case presentation. 13y/o M who developed weight loss, increased LFTs and hyperbilirubinemia. Case presentation (cont.). On liver US: hepatic mass replacing most of left hepatic lobe and involving the periphery of the right hepatic lobe. Dermoplastic small round cell tumor (DSRCT).

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Case presentation

Case presentation

  • 13y/o M who developed weight loss, increased LFTs and hyperbilirubinemia


Case presentation cont

Case presentation (cont.)

  • On liver US: hepatic mass replacing most of left hepatic lobe and involving the periphery of the right hepatic lobe.


Dermoplastic small round cell tumor dsrct

Dermoplastic small round cell tumor (DSRCT)

Karen Estrella H.

PGY-2

September 2010


Objectives

Objectives:

  • Introduction

  • Characteristics of the disease

  • Differential diagnosis

  • Clinical findings

  • Diagnosis

  • Treatment

  • Prognosis


Introduction

Introduction

  • Soft tissue sarcomas represent 10% of all children cancers.

  • DSRCT: very rare type of sarcoma

  • 1st described in 1989 in MSKCC

  • male:female ratio = 5:1

  • Mean age of appearance: 21y/o

  • Associated with aggressive features and a poor prognosis.


Introduction cont

Introduction (cont.)

  • Tumor cells co-express epithelial, mesenchymal and neuronal markers and are thought to originate from a mesothelial or submesothelial progenitor cell with the potential to undergo multilineage differentiation.

    • Also called "mesothelioblastoma".

  • Frequently rises from the peritoneum, but also seen in other surfaces: pleura, ovaries, tunica vaginalis, liver, CNS.


Differential diagnosis

Differential diagnosis

  • Ewing sarcoma

  • Rhabdomyosarcoma

  • Neuroblastoma

  • Synovial sarcoma

  • Lymphoma

  • Ectomesenchymoma

  • Wilms’ tumor

  • Carcinoid tumor

  • Neuroendocrine carcinomas

  • Merkel cell carcinoma

  • Small cell mesothelioma in adults


Clinical findings

Clinical findings

  • Regional

  • Usually: abdominal mass with peritoneal and omental implants

    • Crampy abdominal pain, constipation, weight loss

    • Abdominal distention, jaundice, ascitis

  • Other reported sites of disease include pleura, ethmoid sinuses, scalp, hand, posterior cranial fossa, pancreas, ovary, paratesticular and kidney.

  • Liver metastases are common at diagnosis and relapse; other distant sites include lymph nodes, lung, bone and bone marrow.


Diagnosis

Diagnosis

CT SCAN


Diagnosis1

Diagnosis

PET SCAN


Histology

Histology

  • MACRO:

    • Solid, firm, multilobulated gray-white masses where cystic areas can also be found.

  • MICRO:

    • small cells that can be round, ovoid or spindled usually grouped in clumps, cords, nests or sheets.

  • Immunohistochemical markers :

    • Epithelial: cytokeratin

    • Mesenchymal: desmin, vimentin.

    • Neural: neuron-specific enolase, synaptophysin.

  • CD99, a marker associated with the Ewing’s sarcoma family positive in 23% of cases .


Histology cont

Histology (cont.)

  • The most specific diagnostic tool is the presence of translocation between EWS and WT-1 gene:

    • t(11;22)(p13;q12)

    • detected by RT-PCR and FISH

    • it encodes a binding protein, thought to have roles in both transcription and splicing

  • Related with oncogenenic factors such as:

    • IGF-1 receptor, PDGFα, PAX2-2, WT-1, ENT4, TALLA-1 and IL-2/15Rβ

    • CCN2 (connective tissue growth factor): associated with dissemination


Treatment

Treatment

  • Options include debulking surgery, chemotherapy w/ or w/o stem cell transplantation, radiotherapy, and recently introduced molecularly targeted therapies.

    • Resection of > 90% of tumor + removal of parietal and visceral peritoneum, omentectomy, splenectomy and even removal of diaphragm.


Treatment cont

Treatment (cont.)

*No standard therapeutic regimen described since no modality is clearly superior to any other.


Treatment cont1

Treatment (cont.)

  • Chemotherapy (P6 chromosomes):

    Alkylating sensitive.

    • Cyclophosphamide, doxorubicin, vincristine, ifosfamide, etoposide, for 7cycles.

      • On cycle 4: CT scans for evaluation, consider adding:

    • cisplatin, carboplatin, topotecan, temozolamide, vinorelbine and irinotecan.

  • Invariably relapse: theory of “cancer stem cells”


Case presentation

Vincristine

Vinorelbine

Cyclophosphamide

Doxorubicin

Ifosfamide

Etoposide

Topotecan

Irinotecan

Temozolamide


Treatment cont2

Treatment (cont.)

  • Molecular targeted therapies:

    • Leflunomide (SU101) is a platelet-derived growth factor (PDGF) receptor inhibitor that acts by blocking PDGF-stimulated receptor autophosphorylation and subsequent cell-cycle progression.

    • Sunitinib is a multikinase inhibitor that targets VEGFR 1, 2 and 3, PDGFR- α, PDGFR -ß, KIT, FLT-3, RET and CSF-1.

    • ENT4, a direct transcriptional target of EWS/WT1


Prognosis

Prognosis

  • Sx+RT+P6 chemotherapy: had a 3-year survival of 55% (29/66), compared to 27% in those patients who did not receive all three therapies.


Case presentation cont1

Case presentation (cont.)

  • Diagnosis was done: 2 yrs ago,

  • 09/08: laparoscopic bx, BMA

    • Infiltration to the mesenterium in the portahepatis, peripancreatric tissue and a heterogeneous mass in LUQ.

    • Adenopathy in the aorto-caval space, left iliac chain, left hemipelvis, and a pelvic mass in cul de sac

    • Infiltration of mesenterium in RLQ and superior to bladder.

    • Placement of percutaneousbiliary drains

    • Cycle 1


Case presentation cont2

Case presentation (cont.)

  • 10/08: cycle 2, later bacteremia with E. faecium

  • 11-12/08: cycle 3-4

  • 01/09: exploratory laparotomy with removal of DSRCT, distal pancreatectomy, splenectomy, resection of large rectal mass with coloproctosotomy, appendectomy and ileostomy

  • 02/09: abdominal abscess, which required sacral and left flank drains

    • Cycle 5

    • Increased output from sacral drain: vesicorrectal fistula

    • Placement of suprapubic catheter, and G-tube

  • 03/09: cycle 6

  • 04/09:cycle 7


Case presentation cont3

Case presentation (cont.)

  • 11/09: disease progression

  • 12/09: septisemia due to K. pneumonia ESBL

  • 07/10: worsening coagulopathy and jaundice, replacement of biliary drains, restart of chemotherapy

  • 08/10: fungemia


References

References

  • http://seer.cancer.gov/publications/childhood/softtissue.pdf

  • http://sarcomahelp.org/dsrct.html

  • http://www.cancerindex.org/clinks2d.htm#2208

  • http://www.dsrct.org/

  • http://www.ncbi.nlm.nih.gov/pubmed/15868593

  • http://www.wjgnet.com/1007-9327/15/4212.pdf

  • http://www.drugdevelopment-technology.com/projects/erbitux/images/2s_Cancer_Cell_Cycle.gif


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