Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
 
Users online: 1940  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
Table of Contents 
CASE REPORT
Year : 2017  |  Volume : 62  |  Issue : 3  |  Page : 318-320
Desmoid tumor of rectus abdominis presenting with Grey-Turner's and Cullen's Sign: A report of a rare case


Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana, India

Date of Web Publication12-May-2017

Correspondence Address:
Angoori Gnaneshwar Rao
F12, B8, HIG-2 APHB, Baghlingampally, Hyderabad - 500 044, Telangana
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_168_17

Rights and Permissions

   Abstract 

Desmoid tumor of rectus abdominis presenting with Grey-Turner's and Cullen's sign is rare. Herein, we report desmoid tumor of rectus abdominis in a 64-year-old multiparous female who presented with ecchymosis involving left flank and around the umbilicus. Histopathological examination of biopsy from the tumor confirmed the diagnosis of the desmoid tumor. She was referred to a surgeon for radical resection.


Keywords: Cullen's sign, desmoid tumor, Grey-Turner's sign, radical resection


How to cite this article:
Rao AG, Swathi T, Farheen SS, Kolli A, Hari S, Reddy UD, Deepak K, Jagadevapuram K. Desmoid tumor of rectus abdominis presenting with Grey-Turner's and Cullen's Sign: A report of a rare case. Indian J Dermatol 2017;62:318-20

How to cite this URL:
Rao AG, Swathi T, Farheen SS, Kolli A, Hari S, Reddy UD, Deepak K, Jagadevapuram K. Desmoid tumor of rectus abdominis presenting with Grey-Turner's and Cullen's Sign: A report of a rare case. Indian J Dermatol [serial online] 2017 [cited 2020 Mar 28];62:318-20. Available from: http://www.e-ijd.org/text.asp?2017/62/3/318/206174

What was known?
Certain clinical conditions such as acute pancreatitis, ruptured ectopic, aortic rupture, and coagulopathies are known to manifest with Grey-Turner's and Cullen's sign.



   Introduction Top


Desmoid tumors are uncommon fibrous neoplasms originating from the musculoaponeurotic structures, account for 0.03% of all tumors and 3% of all soft-tissue neoplasms.[1] The term desmoid was coined by Muller in 1838 and is derived from the Greek word desmos, which means tendon like.

Grey-Turner's sign refers to bruising of the area between the last rib and top of the hip and is a sign of retroperitoneal hemorrhage. Cullen's sign is superficial edema and bruising in the subcutaneous fatty tissue around the umbilicus. Causes include acute pancreatitis, ruptured ectopic, aortic rupture, and coagulopathies.


   Case Report Top


A 64-year-old female presented with pain in the left flank for 2 months and bluish discoloration around the umbilicus and left flank of 10-day duration. There was no history of injury or intake of anticoagulants or personal or family history of bleeding diathesis. Examination revealed ecchymosis on the left flank extending onto the front of the abdomen and above the hip (Grey-Turner's sign) [Figure 1] and umbilicus and suprapubic region (Cullen's sign) [Figure 2]. An ill-defined firm, tender swelling of 5 cm × 6 cm was palpable in the left paraumbilical region. The swelling became less prominent on leg raising test. Routine hematological investigations were unremarkable. Blood chemistry was within normal limits, and human immune deficiency virus serology was nonreactive. Abdominal computed tomography showed single well-defined oval intermediate signal intensity lesion arising from the rectus sheath on the left side [Figure 3]a. Fine-needle aspiration cytology from swelling showed the clusters of spindle cells with moderate pleomorphism, suggestive of desmoid tumor [Figure 3]b. Histopathology of biopsy from swelling showed the spindle-shaped cells in fasciculated bundles, most of them with a spindle to oval nuclei [Figure 3]c and [Figure 3]d, confirmative of desmoid tumor. She was referred to a surgeon for radical surgical excision.
Figure 1: (Original) Ecchymosis on the left flank extending onto the front of abdomen and above hip (Grey-Turner's sign)

Click here to view
Figure 2: (Original) Ecchymosis around umbilicus and suprapubic region (Cullen's sign)

Click here to view
Figure 3: (original) (a) Computed tomography abdomen showing single well-defined oval intermediate signal intensity lesion arising from the rectus sheath on the left side. (b) Fine-needle aspiration cytology (H and E, ×100) showing clusters of spindle-shaped cells with moderate pleomorphism. (c) Histopathology (H and E, ×10) showing spindle-shaped cells in fasciculated bundles. (d) Histopathology (H and E, ×40) showing spindle-shaped cells with the spindle to oval-shaped nuclei

Click here to view



   Discussion Top


As per the World Health Organization, desmoid tumors are defined as “clonal fibroblastic proliferations that arise in the deep soft tissues and are characterized by infiltrative growth and a tendency toward local recurrence but an inability to metastasize“. Desmoids are characterized by mutations in the β-catenin gene, CTNNB1, or the adenomatous polyposis coli gene. The exact etiology of the desmoid tumor is not known; however, it has been postulated that the immunologic and hormonal changes in the pregnancy or postpartum play an important role. In addition, the local effect of the mechanical stress from a gravid uterus may also contribute to the occurrence of a desmoid tumor.[2]

It is prevalent among women of reproductive age group commonly occurring between the ages of 25 and 60 years. The abdominal desmoid tumor usually presents with pain; however, presentation of a desmoid tumor with Grey-Turner's sign and Cullen's sign in the index case is unique and rare. The anterior abdominal wall is the most common site of predilection with an incidence of 50%. Similarly, the index case also found to have a desmoid tumor in the anterior abdominal wall. Most abdominal wall desmoids measure between 5 cm and 15 cm in size. The size of desmoid (5/6 cm) in the index case is in concert with the size of most abdominal desmoids. Differential diagnosis of desmoid includes hypertrophic scars, nodular fasciitis, and fibroblastic sarcoma. Desmoid tumors are classified into three groups: within the abdominal wall, intra-abdominal, and extra-abdominal accounting for 25%, 15%, and 65%, respectively.[3] Extra-abdominal desmoid tumors are commonly found in the region of the shoulder girdle, trunk, and lower extremities. Furthermore, desmoid tumors are also known to occur in 10%–15% of patients with familial adenomatous polyposis. The association of intra-abdominal desmoids with familial adenomatous polyposis is known as Gardener's syndrome. However, there was no association with familial adenomatous polyposis in the index case.

Radical resection is the ideal and most effective treatment for small and accessible desmoid tumors such as the index case.[4] However, big desmoid tumors require full thickness surgery and reconstruction with synthetic material. Recurrence occurs in up to 45% of patients which depends on the tumor size and on the resection.[5] Radiation therapy can be used for recurrent disease or as a primary treatment to avoid mutilating surgical resection. Moreover, it may be used preoperatively, postoperatively, or as the only treatment.[6] Various therapeutic agents have been tried in the management of desmoid tumor which includes Tamoxifen, anti-estrogen and Indomethcin, COX-2 inhibitor.[7],[8],[9]

Desmoid tumor presenting with Grey-Turner's sign and Cullen's sign has not been reported in the literature so far, and this may be the first case report.

In conclusion, a desmoid tumor of rectus abdominis should strongly be suspected in patients presenting with Grey-Turner's sign and Cullen's sign. This may be added to the list of existing causes of Grey-Turner's sign and Cullen's sign.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Economou A, Pitta X, Andreadis E, Papapavlou L, Chrissidis T. Desmoid tumor of the abdominal wall: A case report. J Med Case Rep 2011;5:326.  Back to cited text no. 1
[PUBMED]    
2.
Johner A, Tiwari P, Zetler P, Wiseman SM. Abdominal wall desmoid tumors associated with pregnancy: Current concepts. Expert Rev Anticancer Ther 2009;9:1675-82.  Back to cited text no. 2
[PUBMED]    
3.
De Cian F, Delay E, Rudigoz RC, Ranchère D, Rivoire M. Desmoid tumor arising in a cesarean section scar during pregnancy: Monitoring and management. Gynecol Oncol 1999;75:145-8.  Back to cited text no. 3
    
4.
Rohrich RJ, Lowe JB, Hackney FL, Bowman JL, Hobar PC. An algorithm for abdominal wall reconstruction. Plast Reconstr Surg 2000;105:202-16.  Back to cited text no. 4
[PUBMED]    
5.
Nuyttens JJ, Rust PF, Thomas CR Jr., Turrisi AT 3rd. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: A comparative review of 22 articles. Cancer 2000;88:1517-23.  Back to cited text no. 5
    
6.
El-Haddad M, El-Sebaie M, Ahmad R, Khalil E, Shahin M, Pant R, et al. Treatment of aggressive fibromatosis: The experience of a single institution. Clin Oncol (R Coll Radiol) 2009;21:775-80.  Back to cited text no. 6
[PUBMED]    
7.
Hansmann A, Adolph C, Vogel T, Unger A, Moeslein G. High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer 2004;100:612-20.  Back to cited text no. 7
[PUBMED]    
8.
Waddell WR, Gerner RE. Indomethacin and ascorbate inhibit desmoid tumors. J Surg Oncol 1980;15:85-90.  Back to cited text no. 8
[PUBMED]    
9.
Signoroni S, Frattini M, Negri T, Pastore E, Tamborini E, Casieri P, et al. Cyclooxygenase-2 and platelet-derived growth factor receptors as potential targets in treating aggressive fibromatosis. Clin Cancer Res 2007;13:5034-40.  Back to cited text no. 9
[PUBMED]    

What is new?
Desmoid tumor of rectus abdominis presenting with Grey-Turner′s and Cullen′s sign is unique.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (1,269 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed1764    
    Printed19    
    Emailed0    
    PDF Downloaded50    
    Comments [Add]    

Recommend this journal