INTERESTING IMAGE
Appearance of Extraosseous Pelvic Ewing Sarcoma
on Triphasic Bone Scan
Marianne Muckle, MD,* Elham Habibi, MD,* Birgit Simon, MD,Þ Matthias Zipfel, MD,þ
Hans Jürgen Biersack, MD,* and Hojjat Ahmadzadehfar, MD*
Abstract: A 24-year-old man with extraosseous Ewing sarcoma in the pelvis
underwent a triphasic bone scintigraphy to rule out bone metastases and local
bone infiltration before chemotherapy. The bone scintigraphy showed tracer
uptake in the tumor in all 3 phases.
Key Words: extraosseous Ewing sarcoma, bone scintigraphy, SPECT/CT
(Clin Nucl Med 2014;39: 406Y408)
Received for publication September 26, 2013; and revision accepted December 28,
2013.
From the *Departments of Nuclear Medicine, †Radiology, and ‡Internal Medicine,
Oncology, University Hospital Bonn, Bonn, Germany.
Conflicts of interest and sources of funding: none declared.
Reprints: Hojjat Ahmadzadehfar, MD, Department of Nuclear Medicine, Uni-
versity Hospital Bonn, Sigmund-Freud-Str. 25, 53127 Bonn, Germany. Email:
hojjat.ahmadzadehfar@ukb.uni-bonn.de.
Copyright * 2014 by Lippincott Williams & Wilkins
ISSN: 0363-9762/14/3904Y0406
406 www.nuclearmed.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
REFERENCES
1. Tefft M, Vawter GF, Mitus A. Paravertebral ‘‘round cell’’ tumors in children.
Radiology. 1969;92:1501Y1509.
2. Kim HS, Kim S, Min YD, et al. Ewing’s sarcoma of the stomach; rare case of
Ewing’s sarcoma and suggestion of new treatment strategy. J Gastric Cancer.
2012;12:258Y261.
3. Machado L, Al-Hamdani A, Sankhla DK, et al. Extraosseous Ewing sarcoma
of the vagina: a rare entity. Ann Saudi Med. 2013;33:182Y186.
4. Farley J, O’Boyle JD, Heaton J, et al. Extraosseous Ewing sarcoma of the
vagina. Obstet Gynecol. 2000;96:832Y834.
5. Shek TW, Chan GC, Khong PL, et al. Ewing sarcoma of the small intestine.
J Pediatr Hematol Oncol. 2001;23:530Y532.
6. Movahedi-Lankarani S, Hruban RH, Westra WH, et al. Primitive neuro-
ectodermal tumors of the pancreas: a report of seven cases of a rare neoplasm.
Am J Surg Pathol. 2002;26:1040Y1047.
7. Lee H, Cho JY, Kim SH, et al. Imaging findings of primitive neuroectodermal
tumors of the kidney. J Comput Assist Tomogr. 2009;33:882Y886.
8. Cotterill SJ, Ahrens S, Paulussen M, et al. Prognostic factors in Ewing’s tumor
of bone: analysis of 975 patients from the European Intergroup Cooperative
Ewing’s Sarcoma Study Group. J Clin Oncol. 2000;18:3108Y3114.
9. Javery O, Krajewski K, O’Regan K, et al. A to Z of extraskeletal Ewing
sarcoma family of tumors in adults: imaging features of primary disease,
metastatic patterns, and treatment responses. AJR Am J Roentgenol. 2011;197:
W1015YW1022.
10. Carrasco Cubero C, Jimenez Arjona J, Michan Dona A. 43 year old male with
a right pelvic mass. Reumatol Clin. 2012;8:225Y226.
11. Verrill MW, Judson IR, Harmer CL, et al. Ewing’s sarcoma and primitive
neuroectodermal tumor in adults: are they different from Ewing’s sarcoma
and primitive neuroectodermal tumor in children? J Clin Oncol. 1997;15:
2611Y2621.
Clinical Nuclear Medicine & Volume 39, Number 4, April 2014
Clinical Nuclear Medicine & Volume 39, Number 4, April 2014 Appearance of Extraosseous Pelvic Ewing Sarcoma

FIGURE 1. A 24-year-old man underwent abdominal CT because of a blunt abdominal trauma in a football game. The CT scan
showed a 10  8  9 cm large tumor mass in the pelvis (A, white arrow). The tumor shifted the urinary bladder upwards to the right
side (A, white arrowhead). The patient underwent MRI of the abdomen and a thorax CT scan, followed by an open biopsy.
MRI showed the tumor (B, white arrow) without any infiltration of bladder wall (B, white arrowhead) and no abdominal metastases.
There were no lung metastases in the thorax CT scan. The biopsy revealed a peripheral primitive neuroectodermal tumor
(PNET)/Ewing sarcoma (ES) with EWSR1 gene translocation. Before chemotherapy with vincristine, ifosfamide, doxorubicin, and
etoposide (VIDE), the patient received triphasic bone scintigraphy combined with SPECT/CT to rule out bone metastases
and also local bone infiltration. The perfusion (A) and blood pool (D) phases of the pelvis after the injection of 700 MBq 99mTc-MDP
showed a slightly increased perfusion and uptake in the mid-pelvis (black arrows) in the tumor region. A, CT scan, transversal
view; B, MRI, coronal view; C, planar perfusion image, ventral; D, planar blood-pool image, ventral.

* 2014 Lippincott Williams & Wilkins www.nuclearmed.com 407

Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Muckle et al Clinical Nuclear Medicine & Volume 39, Number 4, April 2014

FIGURE 2. Whole-body scintigraphy 2 hours post-injection (A) showed increased uptake in the mid-pelvis in the region of the
known tumor (white arrowhead) and also a focally increased uptake above that (white arrow). For a better evaluation of the
pelvic region regarding bone metastases and infiltration, we performed a SPECT/low-dose CT scan of the region, which showed the
tumor with 99mTc-MDP uptake without any metastases or bone infiltration (B, SPECT/CT, transversal view; C, low-dose CT,
transversal view). The term extraosseous Ewing sarcoma (EES) was introduced in 1969 by Tefft et al.1 EES is a rare and highly
aggressive tumor that may develop in any part of the human body, such as the small bowel, esophagus, vagina, pancreas, or
kidney.2Y7 Of the cases, 90% appear between 5 and 30 years of age and EES is more common in men.8 Twenty-five percent to
50% of patients present with metastatic disease; the 5-year survival rate is approximately 50%.8,9 A combination of surgery,
radiotherapy, and chemotherapy attain an increase in survival and disease-free survival. It manifests as painful swelling and,
sometimes, fever, weight loss, anemia, and leukocytosis9; however, our patient did not suffer from any of these. CT scan showed a
significant shrinkage of the tumor after 6 cycles of chemotherapy until October 2013. After tumor resection, pathology showed
no evidence of residual vital tumor cells. Further radiotherapy will be evaluated. Carrasco Cubero et al10 reported recently a
similar case but in the advanced stage, with infiltration into the adjacent soft tissue and bone. Pelvic disease may be associated
with a worse prognosis, perhaps related to increased tumor bulk and difficulty in full resection.11 EES, like osseous Ewing sarcoma,
is positive in triphasic bone scintigraphy and SPECT/CT is very helpful for evaluating the pelvic region for bone infiltration.

408 www.nuclearmed.com * 2014 Lippincott Williams & Wilkins

Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.