Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 7  |  Issue : 1  |  Page : 91-93

Hemophilic pseudotumor of abdomen: A rare case report


Department of Surgery, CMC and Hospital, Ludhiana, Punjab, India

Date of Submission09-Apr-2019
Date of Decision22-Apr-2019
Date of Acceptance10-May-2019
Date of Web Publication16-Mar-2020

Correspondence Address:
Dr. Amit Vipan Mahajan
Department of Surgery, CMC and Hospital, Ludhiana, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_21_19

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  Abstract 


Hemophilic pseudotumor is one of the rare complications of hemophilia, which occurs due to repetitive bleeding resulting in an encapsulated mass of clotted blood and necrosed tissue. Complications and symptoms arise due to pain and compression of surrounding structures. We present a rare case of pseudotumor of the abdomen in a 35-year-old male with severe hemophilia B. Pseudotumor was treated successfully with excision of tumor.

Keywords: Excision, hemophilia, pseudotumor


How to cite this article:
Luther A, Mahajan AV, Pandey S. Hemophilic pseudotumor of abdomen: A rare case report. Indian J Vasc Endovasc Surg 2020;7:91-3

How to cite this URL:
Luther A, Mahajan AV, Pandey S. Hemophilic pseudotumor of abdomen: A rare case report. Indian J Vasc Endovasc Surg [serial online] 2020 [cited 2020 Jul 11];7:91-3. Available from: http://www.indjvascsurg.org/text.asp?2020/7/1/91/280662




  Introduction Top


Hemophilia is an X-linked recessive disorder caused by a lack of coagulation factors. Hemophilia A occurs due to deficiency of Factor VIII, while hemophilia B occurs due to deficiency of Factor IX or Christmas factor. Classical presentation of the hemophilia is more common with a prevalence of 1:10,000, while hemophilia B or “Christmas disease” is found in 1:50,000–1:100,000 individuals.[1] Hemophilic pseudotumor is one of the rare complications of hemophilia. Hemophilic pseudotumor occurs in 1%–2% of patients with severe coagulation disorder.[2] It occurs because of repeated episodes of bleeding at bone fracture site, subperiosteal hemorrhage, or bleeding into the soft tissue. Inadequate resorption results of extravasation of blood result an encapsulated mass of clotted blood and necrosed tissue. With repeated hemorrhagic episodes, these lesions expand over time and cause symptoms because of mass effect.

The majority of all reported hemophilic pseudotumors usually involves the soft tissue, the long bones of lower extremities and pelvis, and rarely occurs in the orbit, mandible, maxilla, clavicle, tibia, radius, humerus, or small bones. A very few intra-abdominal pseudotumors have been reported. In this case report, we describe a patient with massive intra-abdominal pseudotumor.


  Case Report Top


A 35-year-old male diagnosed as hemophilia B at the age of 10 years, presented with complaint of pain in the left iliac fossa and left upper thigh.

On examination, a mass of 15 cm × 15 cm was palpated in the left lower quadrant of the abdomen, firm in consistency with well-defined border. Contrast-enhanced computed tomography (CT) of the abdomen [Figure 1] and [Figure 2] showed a large well-defined heterogeneous density nonenhancing mass, epicentered in the left iliopsoas, gluteal region, and hemipelvis with destruction of the ipsilateral iliac bone. The patient was taken for laparotomy with surgical findings that included a cyst-like tumor of 30 cm × 25 cm on the left side of the abdomen. Mass was compressing and displacing the left ureter, urinary bladder, sigmoid colon, and few small bowel loops to contralateral side with no evidence of any major feeding vessel. Tumor had eroded the ilium bone and extended into the gluteal area in a dumbbell fashion. The well-encapsulated tumor was excised and sent for histopathological examination.
Figure 1: Three-dimensional film with arrow showing pseudotumor eroding into the bony pelvis

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Figure 2: Three-dimensional computed tomography film showing pseudotumor

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He was supported with adequate factors preoperatively and intraoperatively. There was no immediate or late complication postsurgery. Biopsy report was consistent with diagnosis of hemophilic pseudotumor. A follow-up ultrasound done 2-week postsurgery showed no new lesion at site of the excised mass.


  Discussion Top


The hemophilic pseudotumor was first described by Starker, who described the entity in femur of a 14-year-old boy.[3] Hemophilic pseudotumor is observed only in 1%–2% of patients with severe disease. Now hemorrhagic episodes are well controlled with factor substitution therapy, because of which incidence of pseudotumor may have decreased. However, factor substitution therapy is ineffective in patients with autoantibodies against Factor VIII or Factor IX.[4]

Most of the morbidity from pseudotumors is due to their compressive effect on surrounding structures, including bone destruction, muscle necrosis, and skin necrosis. In our case report, the patient presented with pain in the left iliac fossa and left upper thigh. Erosion into an artery may also be responsible for massive bleeding. Compartment syndromes occur due to vascular compromise and joint contractures. Pseudotumor distorts the anatomy by causing major displacement of the bowels, ureters, nerves, and blood vessels and thus increasing the likelihood of injury during surgery.[5] Hemophilic pseudotumor is diagnosed by histological examination and various imaging modalities. On histological examination, hemophilic pseudotumor resembles hematoma with a dense fibrous capsule. The cyst wall is formed by collagenous connecting tissue and cavity contains a variable amount of organized fibrous tissue, thick “toothpaste-like” debris, and liquefied clots.[6] CT scan is particularly helpful in the evaluation of bone, whereas magnetic resonance imaging is superior to CT scan for soft-tissue imaging. CT scan delineates the size and extent of the tumor and compression of the adjacent skeleton, tissue, and organs. CT scans have been proven to be particularly efficient in detection of daughter cysts, deep-seated cysts, and tumor extension into adjacent tissues. Ultrasonography can be used after surgery to monitor for recurrence of pseudotumor in soft tissue.[7]

Various treatment modalities are available for hemophilic pseudotumor depending on location and duration of the tumor. Conservative approach is done for pseudotumor caused by recent hemorrhage, which includes factor replacement and immobilization of affected area. Current literature indicated that surgical excision of pseudotumor is the recommended treatment.[8] Based on the outcome obtained in our case, we stress the advantage of the surgical option as one of the treatment modalities.

Pseudotumor excision should start in healthy tissue in which anatomy is clearly identified. Pseudotumor should be excised en bloc or as completely as possible. The effect of surgery is better when the pseudotumor is small. Risk of postoperative complications is that there are vessel and nerve damage, fistula formation, pseudotumor recurrence, abscess, coagulation factor inhibitors formation, and even death. Surgical resection after performing arterial embolization to reduce the vascularization of pseudotumor is a good alternative.

Radiation therapy has been indicated in those cases in which an inhibitor factor has developed postoperatively. Hence, adequate factor replacement and assessment of any factor that inhibits coagulation factor level should be maintained at 100% level for 3 days after surgery and then at 50% in the next 2 weeks.[9]

In our case, we successfully removed the intra-abdominal mass completely and the patient was supported with adequate factor support. The patient was being assessed with ultrasound every 2 weeks for initial 3 months and is now being followed 6 monthly in the outpatient department. The patient is doing well with no radiological recurrence.


  Conclusion Top


Hemophilic pseudotumor is well-documented complication of bleeding disorders that surgeon should be aware of while dealing with such patients. Signs of compression should be evaluated early because its diagnosis is crucial for evaluation and proper surgical planning and treatment in conjunction with other specialist. The surgical resection of the hemophilic tumor is a complex and demanding procedure with unpredictable postoperative course including many possible complications, requiring detailed preoperative planning. One should be aware of unpredictable outcome of this complex procedure. To minimize possible complications, we recommend the surgical treatment of the hemophilic pseudotumor in multispecialty centers with close cooperation between all disciplines. Patients should be educated about aseptic self-administration of factor, be informed of a possible failure and complications and be monitored at regular intervals. Surgical treatment of the hemophilic pseudotumor is feasible and safe during the intraoperative and postoperative course, when performed in specialized centers.

To conclude, patients with advance disease and complications might benefit with a conservative approach, but further decision-making would need a multidisciplinary approach.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Koenigkam-Santos M, Polezi MB, Engel EE, Pastorella MT, Simao MN, Elias J Jr., et al. Uncommon presentations of intraosseous haemophilic pseudotumors in imaging diagnosis. Radiol Bras 2009;42:159-63.  Back to cited text no. 1
    
2.
Ahlberg AK. On the natural history of hemophilic pseudotumor. J Bone Joint Surg Am 1975;57:1133-6.  Back to cited text no. 2
    
3.
Magallon M, Monteagudo J, Altisent C, Ibanez A, Rodriguez-Perez A, Riba J, et al. Hemophilic pseudotumour: multicenter experience over a 25-year period. Am J Hematol. 1994; 45:103-8.  Back to cited text no. 3
    
4.
Lee CA, Berntorp EE, Hoots WK, editors. Textbook of Haemophilia. 2nd ed. Hoboken: Wiley-Blackwell; 2010.  Back to cited text no. 4
    
5.
Coon WW, Penner JA. Management of abdominal hemophilic pseudotumor. Surgery 1981;90:735-40.  Back to cited text no. 5
    
6.
Magallon M, Monteagudo J, Altisent C, Ibanez A, Rodriguez-Perez A, Riba J, et al. Haemophilic pseudotumor: Multicenter experience over a 25-year period. Am J Hematol 1994;45:103-8.  Back to cited text no. 6
    
7.
Pettersson H, Ahlberg A. Computed tomography in hemophilic pseudotumor. Acta Radiol Diagn (Stockh) 1982;23:453-7.  Back to cited text no. 7
    
8.
Espandar R, Heidari P, Rodriguez-Merchan EC. Management of haemophilic pseudotumours with special emphasis on radiotherapy and arterial embolization. Haemophilia 2009;15:448-57.  Back to cited text no. 8
    
9.
Rodriguez-Merchan EC. The haemophilic pseudotumor. Haemophilia 2002;8:12-6.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2]



 

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