Reccurent desmoid tumour of the chest wall

Clinical History

A 45-year-old female patient with a history of surgically resected lipoma and desmoid tumour in the right chest wall, noticed a progressively growing lesion at the area of the scar two years later. Physical examination revealed a firm and fixed mass, the skin was movable with no sign of inflammation.

Imaging Findings

Ultrasound of the right chest wall demonstrated a large hypoechoic mass in the subcutaneous tissue, poorly vascularized at colour Doppler (Fig. 1).

Contrast-enhanced CT showed a homogeneous tumour of tissue density in the right chest wall, with irregular contours in some areas and lack of enhancement, invading the right serratus muscle, measuring 121x42 mm. The adjacent subcutaneous tissue, bone and the right pulmonary parenchyma were intact (Fig. 2).

On chest MRI, the tumour was seen invading the right serratus muscle, demonstrating isosignal T1 intensity, low T2 signal intensity and enhancement post gadolinium administration without vascular compression (Fig. 3).

CT guided trans-parietal biopsy before surgical resection and pathological result confirmed a desmoid tumour measuring 13 × 4 cm.

Discussion

As described previously by MacFarlane in 1832 [1], desmoid tumour (aggressive fibromatosis) is an uncommon soft tissue tumour characterized by proliferation of fibroblasts and myofibroblasts. Women are most affected [2], and it frequently occurs during the second or third decades of life. [3]

Desmoid tumours are associated with a history of traumatic or post-surgical scar in the site [2], high-oestrogen states or familial adenomatous polyposis (FAP ) [3], they are also characterized by their local invasive behaviour and frequent recurrences without metastatic potential [1]. Abdominal wall is the most frequent localization, but involvement of the chest wall is present in only 10 to 28% of the patients [2], that is usually characterized by a palpable mass (5 to 10 cm, rarely larger than 20 cm [1]) and pain secondary to the nerve involvement, while the intrathoracic involvement is exceptional [1].

Imaging allows to precisely define the degree of local extension. Chest X-ray can show a parietal opaque mass that has invaded the adjacent soft and bone tissues. Ultrasound is not specific [1]. On computed tomography, desmoid tumours have a variable attenuation similar to that of muscle with possibility of enhancement after contrast administration [2]. It usually reveals the size, location, and reaction of the adjacent bone; however, the soft tissue infiltration and the local recurrence are precisely detected on MR imaging [4], which shows a signal intensity similar to that of muscle on T1, and variable signal intensity on T2 [2].

The final diagnosis requires histological assessment but the transparietal biopsy may not be sufficient because of the relative low cellularity of the tumour [2-5].

The principal therapeutic manoeuvre of the desmoid tumours is surgical excision [6] and radiation (indicated when the surgical excision cannot be performed) [5]; however, extra-abdominal desmoid tumours have a high local recurrence rate after resection [5].

Our patient did not have a typical location (chest wall) and age of presentation (45 years old), but the size, slowly growing character and the local recurrence after resection of the tumour (frequent in the extra-abdominal localization [5]) were consistent with desmoid tumour. In our case, CT-guided transparietal biopsy confirmed the diagnosis of recurrent desmoid tumour.

Differential Diagnosis List

Final Diagnosis

Reccurent desmoid tumor of the chest wall

URL:	https://www.eurorad.org/case/11659
DOI:	10.1594/EURORAD/CASE.11659
ISSN:	1563-4086