0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Special Feature |

Image of the Month—Diagnosis FREE

[+] Author Affiliations

Section Editor: Carl E. Bredenberg, MD

More Author Information
Arch Surg. 2010;145(2):206. doi:10.1001/archsurg.2009.279-b.
Text Size: A A A
Published online

ANSWER: SCLEROSING ANGIOMATOID NODULAR TRANSFORMATION OF THE SPLEEN

The removed spleen measured 9 × 7 × 5 cm and weighed 133 g. Grossly, the lesion was a single, well-circumscribed, round-to-bosselated mass that measured 4 × 3.5 cm (Figure 2). At its periphery, multiple dark brown nodules were interspersed with stellate whitish fibrotic stroma. On microscopic examination, multiple angiomatoid nodules were seen submerged in a fibrotic stroma, with sharp demarcation of the lesion from the adjacent splenic parenchyma (Figure 2). The angiomatoid nodules were composed of complex disorganized vasculature with 3 distinct types of vessels that showed immunostain reactivity to CD34, CD8, and CD31. The lesion was consistent with sclerosing angiomatoid nodular transformation (SANT).

Place holder to copy figure label and caption
Figure 2

Cut surface of the spleen showing a bosselated mass with multiple dark brown nodules interspersed with stellate whitish fibrotic stroma.

Graphic Jump Location

Sclerosing angiomatoid nodular transformation of the spleen is an entity recently described by Martel et al1 and earlier under the term capillary cord hemangiomas by Krishnan et al.2 It is usually asymptomatic, seen in women in middle age, and diagnosed on imaging studies performed on patients for other reasons, usually in a background of malignancy. Patients may present to the surgeon with abdominal pain, raised erythrocyte sedimentation rate, fever, and anemia, requiring an extensive evaluation.

Hemangioma is the most common vascular tumor of the spleen and is distinguished from SANT by nuclear imaging. Histologically, it is composed of a single type of blood vessel similar to littoral cell angioma and lacks the distinctive angiomatoid nodular features seen in SANT.3 Epithelioid hemangioendothelioma is a rare splenic tumor of borderline malignant potential. It may be differentiated from SANT by the presence of cytological atypia and a low mitotic index.4

Splenic hamartomas contain structurally disorganized red pulp tissue and are differentiated from SANT by the absence of angiomatoid nodular pattern. Metastases to the spleen may also present as multiple nodules owing to exaggerated stromal response and are easily differentiated on histology. Kaposi sarcoma is usually seen in immunocompromised patients with human immunodeficiency virus or after transplant.

Splenectomy is uniformly curative in all patients with SANT. No recurrence has been reported to date.

Return to Quiz Case.

Submissions

The Editor welcomes contributions to the Image of the Month. Manuscripts should be submitted via our online manuscript submission and review system (http://manuscripts.archsurg.com). Articles and photographs accepted will bear the contributor's name. Manuscript criteria and information are per the Instructions for Authors for Archives of Surgery (http://archsurg.ama-assn.org/misc/ifora.dtl). No abstract is needed, and the manuscript should be no more than 3 typewritten pages. There should be a brief introduction, 1 multiple-choice question with 4 possible answers, and the main text. No more than 2 photographs should be submitted. There is no charge for reproduction and printing of color illustrations.

Correspondence: Shankar R. Raman, MD, MRCS, 1770 Grand Concourse 12L, Bronx, NY 10457 (shankarrraman@gmail.com).

Accepted for Publication: March 19, 2009.

Author Contributions:Study concept and design: Raman. Acquisition of data: Raman. Analysis and interpretation of data: Raman, Parithivel, and Niazi. Drafting of the manuscript: Raman. Critical revision of the manuscript for important intellectual content: Parithivel and Niazi. Administrative, technical, and material support: Raman, Parithivel, and Niazi. Study supervision: Parithivel and Niazi.

Financial Disclosure: None reported.

Martel  MCheuk  WLombardi  LLifschitz-Mercer  BChan  JKRosai  J Sclerosing angiomatoid nodular transformation (SANT): report of 25 cases of a distinctive benign splenic lesion. Am J Surg Pathol 2004;28 (10) 1268- 1279
PubMed Link to Article
Krishnan  JDanon  AFrizzera  D Use of anti-factor VIII-related antigen (F8) and QBEN10 (CD34) antibodies helps classify the benign vascular lesions of the spleen. Mod Pathol 1993;694A
Arber  DAStrickler  JGChen  YYWeiss  LM Splenic vascular tumors: a histologic, immunophenotypic, and virologic study. Am J Surg Pathol 1997;21 (7) 827- 835
PubMed Link to Article
Kutok  JLFletcher  CD Splenic vascular tumors. Semin Diagn Pathol 2003;20 (2) 128- 139
PubMed Link to Article

Figures

Place holder to copy figure label and caption
Figure 2

Cut surface of the spleen showing a bosselated mass with multiple dark brown nodules interspersed with stellate whitish fibrotic stroma.

Graphic Jump Location

Tables

References

Martel  MCheuk  WLombardi  LLifschitz-Mercer  BChan  JKRosai  J Sclerosing angiomatoid nodular transformation (SANT): report of 25 cases of a distinctive benign splenic lesion. Am J Surg Pathol 2004;28 (10) 1268- 1279
PubMed Link to Article
Krishnan  JDanon  AFrizzera  D Use of anti-factor VIII-related antigen (F8) and QBEN10 (CD34) antibodies helps classify the benign vascular lesions of the spleen. Mod Pathol 1993;694A
Arber  DAStrickler  JGChen  YYWeiss  LM Splenic vascular tumors: a histologic, immunophenotypic, and virologic study. Am J Surg Pathol 1997;21 (7) 827- 835
PubMed Link to Article
Kutok  JLFletcher  CD Splenic vascular tumors. Semin Diagn Pathol 2003;20 (2) 128- 139
PubMed Link to Article

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

See Also...
Articles Related By Topic
Related Collections
PubMed Articles