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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

  • 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)

Karen Estrella H.

PGY-2

September 2010


Objectives:

  • Introduction

  • Characteristics of the disease

  • Differential diagnosis

  • Clinical findings

  • Diagnosis

  • Treatment

  • Prognosis


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.)

  • 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

  • Ewing sarcoma

  • Rhabdomyosarcoma

  • Neuroblastoma

  • Synovial sarcoma

  • Lymphoma

  • Ectomesenchymoma

  • Wilms’ tumor

  • Carcinoid tumor

  • Neuroendocrine carcinomas

  • Merkel cell carcinoma

  • Small cell mesothelioma in adults


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

CT SCAN


Diagnosis

PET SCAN


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.)

  • 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

  • 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.)

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


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”


Vincristine

Vinorelbine

Cyclophosphamide

Doxorubicin

Ifosfamide

Etoposide

Topotecan

Irinotecan

Temozolamide


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

  • 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 (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 (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 (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

  • 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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