BILATERAL GIANT INTRAMUSCULAR HEMANGIOMA OF THE BUTTOCKS: AN UNUSALL DIAGNOSIS IN AN 80 YEAR-OLD MAN.

ABID Bassem | REJAB H | FENDRI S | MEJDOUB Y | AFFES N | BEN AMEUR H | BEN AMAR M | BOUJELBEN S |

La tunisie chirurgicale - 2022 ; Vol 2022

Resumé

Hemangiomas are a rare, benign vascular tumors derived from epithelial cell lines. Intramuscular hemangiomas (IMHs) are infrequent, representing less than 1% of all hemangiomas. Diagnosis of this lesion can be difficult especially for older patients, due to its rarity and nonspecific clinical presentation.Ultrasound, computed tomography scans, and magnetic resonance imaging studies can help to define the precise location and size of IMHs prior to treatment. Complete surgical excision is the treatment of choice. We report a case of an 80 year-old man who presented a bilateral intramuscular hemangioma which was successfully managed surgically. Trough our case and a brief review of the literature, we try to focus on histological particularities, diagnosis and therapeutic modalities of this rare entity.

 

Mots Clés

Intramuscular hemangiomas

Introduction :

Hemangiomas are a rare benign vascular tumors derived from epithelial cell lines. Intramuscular hemangiomas (IMHs) are infrequent, representing less than 1% of all hemangiomas. Diagnosis of IMH can be difficult due to its rarity and nonspecific clinical presentation. We report a case of an 80 year-oldman who presented a bilateral intramuscular hemangiomawich was successfully managed surgically. Trough our case and a brief review of the literature, we try to focus on histological particularities, diagnosis and therapeutic modalities of this rare entity.

Article

Case Report:

            An 80-year-old man was admitted to the department of general surgery for a slowly growing palpable mass of the buttocks evolving since 3 years reaching a very important size and causing functional discomfortespecially in thesitting position.Clinical examinationfound twoswellings, the first one, located in the right buttock, was soft in consistency, painless and measuring 16/12 cm in size(fig 1). The second one, located in the left buttock, was also painless but firm and making 7/4cm in diameter (fig 2).Thrills, bruit, pulsation or skin discolorations were absent. There was no previous history of trauma.Ultrasonographyshowed in the right side, awell limitedliquid massof16.5/12cmwithheterogeneous and denseechogenic areas(fig 3), and in the left side a calcified well limited mass.The computed tomography (CT) scan didnot provide anyadditional information (fig 4). The diagnosis of soft tissue hydatid cysts was then retained regarding the clinical presentation and radiological findings of these lesions and with a view that we represent an endemic area of hydatid cysts.

The patient was operated by local approach. Exploration per operatively revealed in the right gluteal region, a well encapsulated fluid mass depending on the glutealmuscles butdoesnot contain amembraneorhydatid sand (fig 5). However, the left mass has a tissular consistencyand seat ofmultiple calcifications (fig 6).Complete surgical excision with clear-cut margins was then performed for the two lesions (fig 7).

Histological examination has given the final diagnosis of geant right intra muscular hemangioma and thrombosed and totally necrosed left hemangioma. The patient was discharged on the fourth postoperative day and his still asymptomatic with no recurrence after two years of follow-up.  

Discussion:

Hemangiomas are a rare, benign vascular tumours derived from epithelial cell lines [1, 2]. They represent the most frequent benign vascular neoplasm during infancy.Hemangiomas are most frequently located on the head and neck (80%), followed by the trunk and extremities [2]. Liston has described the first case of hemangioma in 1843 and called it “erectile tumour”[1]. Intramuscular hemangiomas (IMHs) are infrequent, representing less than 1% of all hemangiomas [3]. The most common location of IMH is the lower extremities (45%), followed by the upper extremities (27%) and head and neck (14%)(2).The exactetiology of this lesion is unknown. On the one hand, it is believedto develop from abnormal embryonal sequestrations [1]; Thishypothesisis supported by thegiventhat thirty per cent of lesionsappear atbirth up, while the majority of the remainingcases appear during the first few weeks after birth [2]. On the other hand, muscular trauma has been proposed as an initiating factor explaining why most of them occur in young populationwith 94% occurring before the age of 30 but it can occur in patients of all ages [1]. We think that our patient is the oldest one in the literature. The sizeof the tumourmay range from less than 4 cm to greater than 20 cm, but most are less than 9 cm in diameter [4].

The histological appearance of the lesion is variable. These lesions are most frequently non-encapsulated benign neoplasms composed of differently sized vascular channels [1]. So that Allen and Enzinger[5] have classified them into five categories, depending on the predominant type of vascular channel identified: capillary, cavernous,arteriovenous, venous, and mixed variations. In addition to their vascular components, hemangiomas can contain a thrombus, calcifications and admixed with variable amounts of adipose, fibrous and myxoid tissues. There may be evidence of inflammation and fibrous proliferation [1, 4].  Malignant transformation or spontaneous regression generally does not occur, thereforesurgical intervention may be required.

Clinically, the lesions usually present as slowly growing soft tissue masses with few symptoms. Thrills, bruit, pulsation or skin discolorations, usually associated with superficial haemangiomas, are absent. Consistency may vary from soft and diffuse to firm and localized due to their deep location [1, 3, 4]. In cases of lesions located in the lower limbs, pain during physical activity is the commonest symptom, in fact, the increased blood flow into the vascular spaces of the haemangioma causesexpansion of the lesion during times ofphysical activity and which become consistent and painful. Occasionally, tenderness is present over the site of the lesion. Pulsations, bruits and thrills are unusual [6].

Diagnosis of IMH represents a challenge for both clinician and radiologist due to its rarity and nonspecific clinical presentation. Plain radiographs may show nonspecific changes such as phleboliths or periosteal reaction of adjacent bone [7].

Colourdoppler sonography is useful to demonstrate the vascular structures in and around the muscle [1]. Computed tomography(CT) scan is very useful in the evaluation of IMH: it reveals the involvement of adjacent structures and identifies focal calcifications (phleboliths) present in approximately 25% of cases [3]. IMH often remains undiagnosed preoperatively, as in our case. However, if practiced, Magnetic Resonance Imaging (MRI) is a reliable tool in detecting and delineating deep seated and large IMH by showing high signal intensity on most T1- and T2-weighted images [1,7]. Angiography is usually of little value except in case of large haemangioma where preoperative embolization would be of use in decreasing the size of the lesion [6]. Fine needle aspiration biopsy is contraindicatedbecause of the risk of bleeding [2].

The main differential diagnosis is arterial malformations. The latter usually have a thrill and murmur on auscultation, and a feeding artery is evident during arteriogram [2]. The others differential diagnoses include lipoma, fibroma, enlarged lymph nodes, compartment syndrome, hematoma, hernia, and soft-tissue sarcoma [7].

Surgical excision is the treatment of choice for intramuscular haemangiomas [1-3, 6]. Complete surgical excision with clear-cut margins is the best therapeutic approach, although every patient with IMH should be treated individually with consideration given to tumour location, surgical accessibility, depth of invasion, patient’s age, and cosmetic factors[3, 7]. Radiotherapy, cryotherapy, embolization, steroid injection, laser therapy, and injection of sclerosing agents may be beneficial in case of impractical and/or incomplete surgical excision[1, 3, 7] despite their limited rate of success in the literature [6].

Although the benign nature of these lesions, they are associated with local recurrence rate up to 20% which is thought to be related to inadequate primary surgical excision rather than histological subtypes[1, 6, 7].

 

Conclusion:

Intramuscular hemangioma is a rare benign tumour which represents a challenge in diagnosis especially for older patients. Ultrasound, computed tomography scans, and magnetic resonance imaging studies can help to define the preciselocation and size of intramuscular hemangiomas prior to treatment. Surgical excision remains the treatment of choice. The prognosis is dominated by the risk of recurrence.

Références

[1]. Arun B N, Manjula B V, Balasubramanyam A M. Intramuscular haemangioma of mylohyoid muscle: A case report. Indian J Surg 2010; 72 (1): 344-346.

[2]. Ranero-Juarez A G, Rosales-Galindo V M, Leon-Takahashi A M, Arenas-Guzman R, and Carlos Garcia. Intramuscular hemangiomas of the extremities: report of six cases. International Journal of Dermatology 2009; 48: 875-878.

[3]. Griffo S, Stassano P, De Luca G, Di Tommaso L, Monaco M, and Spiezia S. Intramuscular hemangioma of the chest wall: An unusual tumor. J Thorac Cardiovasc Surg 2007; 134: 1368-9.

[4]. Jin W, Kim G Y, Lee J H, Yang D M, Kim H C, Park J S, Ryu K N. Intramuscular Hemangioma With Ossification. J Ultrasound Med 2008; 27: 281–285.

[5]. Allen PW, Enzinger FM. Hemangioma of skeletal muscle: an analysis of 89 cases. Cancer 1972; 29: 8-22.

[6]. Quinn P S, Sieunarine K, Lawrence-Brown M and Tan P. Intramuscular haemangiomas: hookwire localization prior to surgical excision: report of four cases. ANZ J. Surg 2001; 71: 62-66.

[7]. Wisniewski SJ, Newcomer K, and Stanson AW. Intramuscular Hemangioma of the Foot: A Diagnostic Dilemma. Med. Sci. Sports Exerc 2005; 37 (10): 1655–1657.

 

Figure 1:Clinical examination: mass located in the right buttock measuring 16/12 cm.

 

Figure 2: Bilateral masses of the buttocks on physical examination.

 

 

Figure 3:Ultrasonography: awell limitedliquid mass in the right buttockof16.5/12cmwithheterogeneous and denseechogenic areas.

 

Figure 4: Computed tomography: bilateral masses on the gulteal region.

 

 

 

 

Figure 5: Exploration per operatively: a well encapsulated fluid mass depending on the rightglutealmuscles.

 

Figure 6:Exploration per operatively: left mass with tissular consistencyand seat ofmultiple calcifications.

 

 

 

 

Figure 7:Complete surgical excision with clear-cut margins for the two lesions.