Desmoplastic small round cell tumors of the paratesticular region

A report of six cases

Oscar Cummings, Thomas Ulbright, Robert H. Young, Angelo P. Dei Tos, Christopher D M Fletcher, Meredith T. Hull

Research output: Contribution to journalArticle

117 Citations (Scopus)

Abstract

Desmoplastic small round cell tumor (DSRCT) typically occurs in the abdomen but may also present at other sites. We report six cases of paratesticular DSRCT. The patients, who ranged in age from 17 to 37 (mean, 28) years, presented with a scrotal mass (five cases) or testicular pain (one case). Grossly, the tumors were white to tan and firm. Typically, they involved the paratesticular soft tissue, serosal surfaces and the epididymis near the junction with the rete testis. Microscopically, the tumors consisted of nests of mitotically active 'small blue cells' with scant cytoplasm embedded in a densely fibrotic stroma. Two tumors showed focal tubule formation; one of these also formed rosettes. The tumors exhibited the typical immunophenotype of DSRCT (positivity for keratin, vimentin, desmin, and neuron-specific enolase but nonreactivity with HBA-71 and anti-S-100). Four tumors metastasized to lymph nodes (retroperitoneal, cervical, and two unspecified); pulmonary metastases occurred in one of these cases and in one patient without lymph node metastases. One of the above patients treated with chemotherapy, died of disease at 16 months. The patterns with pulmonary metastases (one of whom also had lymph node metastases) were treated with aggressive chemotherapy and are alive and apparently disease-free at 2.5 and 3 years, respectively. Three of the six patients, two of whom had known metastases, were lost to follow-up. The DSRCT of the paratestis has histologic and immunohistochemical features identical to its abdominal counterpart and must be differentiated from other 'small blue cell' tumors of the paratesticular region.

Original languageEnglish
Pages (from-to)219-225
Number of pages7
JournalAmerican Journal of Surgical Pathology
Volume21
Issue number2
DOIs
StatePublished - 1997

Fingerprint

Desmoplastic Small Round Cell Tumor
Neoplasm Metastasis
Neoplasms
Lymph Nodes
Rete Testis
Drug Therapy
Lung
Desmin
Phosphopyruvate Hydratase
Epididymis
Lost to Follow-Up
Vimentin
Keratins
Abdomen
Cytoplasm
Pain

Keywords

  • Desmoplastic small round cell tumor
  • Immunohistochemistry
  • Paratesticular neoplasm

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine

Cite this

Desmoplastic small round cell tumors of the paratesticular region : A report of six cases. / Cummings, Oscar; Ulbright, Thomas; Young, Robert H.; Dei Tos, Angelo P.; Fletcher, Christopher D M; Hull, Meredith T.

In: American Journal of Surgical Pathology, Vol. 21, No. 2, 1997, p. 219-225.

Research output: Contribution to journalArticle

Cummings, Oscar ; Ulbright, Thomas ; Young, Robert H. ; Dei Tos, Angelo P. ; Fletcher, Christopher D M ; Hull, Meredith T. / Desmoplastic small round cell tumors of the paratesticular region : A report of six cases. In: American Journal of Surgical Pathology. 1997 ; Vol. 21, No. 2. pp. 219-225.
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