Management of symptomatic dorsal myofibromatosis: a case report and review of the literature

Dorsal fibroma (ED) is a rare tumor that occurs most often in the subscapularis and subscapular region between the chest wall, the anterior dentate muscle, and the latissimus dorsi muscle. Based on a literature review, ED was considered an extremely rare entity. However, the incidence may be greater and more difficult to determine because the majority of ED cases are asymptomatic and therefore undiagnosed. Surgical excision is usually performed when patients experience pain associated with erectile dysfunction. In this case, it is important to evaluate the contributing factors to the pain seen in these patients and to weigh the risks versus benefits of the intervention in asymptomatic ED patients who present for potential surgical intervention. Here we report a case of bilateral erectile dysfunction, located in the upper back with only the right side of symptoms in a 56-year-old worker. Due to pain in the right upper back, the patient underwent surgery to remove ED. The postoperative course was uneventful and the patient made an excellent recovery. A review of the literature showed no relationship between pain at presentation and tumor size or location. The main complications of treatment in these patients include the formation of a seroma or hematoma which can be avoided according to the literature with the use of a postoperative drainage tube and compression dressings.

an introduction

Dorsal fibroma (ED) is a rare tumor that occurs most often in the subscapularis and subscapular region between the chest wall, the anterior dentate muscle, and the latissimus dorsi muscle. [1]. ED is traditionally considered a pseudotumor caused by repetitive mechanical stress between the tip of the scapula and the chest wall resulting in microtrauma and an overproduction of elastic tissue from stimulating fibroblasts. Thus, the relationship between manual/heavy activities and the development of ED, as in the case of our patient, was investigated. Based on the existing literature, ED has been considered a very rare condition, however, the incidence may be greater and difficult to identify with the majority of ED cases being asymptomatic and thus undiagnosed. [2]. However, it is important to weigh the risks against the benefits of intervention in asymptomatic ED patients who present for potential surgical intervention.

Here we report a case of bilateral erectile dysfunction, with symptoms on the right side only, in a 56-year-old worker who underwent surgical resection of the right side of the upper back. We conducted a literature review to investigate potential factors causing ED to be symptomatic and to discuss potential surgical interventions due to the risk of complications.

View status

A 56-year-old man presented with a painful lump in the upper right back that had gradually enlarged over the past two years. He had acute, non-radiating pain at the site of the mass, which he rated as seven out of ten on the pain scale. The patient had a previous history of a back injury that caused radiating back pain. He had no history of recent/new injury at the time of his presentation. The pain at the site of his lumps was different from his chronic pain. The patient reported repetitive manual labor in his job as a construction worker that constantly involved lifting heavy objects.

On physical examination, there was an 8 cm mass in the right upper back that was sensitive to palpation. The patient did not show any neurological signs. Range of motion (ROM) of the upper limbs and shoulders remained bilaterally intact, with some mild discomfort upon abduction and adduction of the right shoulder.

Examination by computed tomography of the chest with intravenous contrast showed bilateral soft tissue masses located in the upper back directly in the trapezius depth, latissimus dorsi and posterior dentate muscles (Fig. 1). On the right, the block measures 7.3 x 3.1 x 6.5 cm. On the left, the block measures 6.8 x 1.3 x 5.6 cm. CT scan results consistent with ED have been reported

Due to the patient’s persistent right-sided pain, discomfort from over-the-counter medications, and the nature of his function, the patient requested surgical removal of the right-sided mass to relieve pain and discomfort.

Surgical excision was performed under general anaesthesia. The mass was found deep in the deep fascia and was noted to be muscular, measuring approximately 6 x 6 cm. Prior to wound closure, microhemostasis was obtained, and a Blake drain was placed deep into the muscle cavity to prevent hematoma or seroma formation. The wound was closed in three layers.

The sample was sent for pathological evaluation. The pathology report stated that the lesion was composed of fibrous tissue that showed an admixed bland spindle cell component as well as some adipose tissue. Trichromium staining showed extensive collagen deposition and elastic tissue patches showed irregular dense clumps of elastic material. The sample was highly positive for vimentin, with smooth muscle actin, desmin, and s100 being negative. CD34 staining showed some positive cells and p53 staining showed some weak positivity. Based on these findings, the pathology determined that these features were consistent with the clinical presentation of fibrosarcoma, and no metastasis was identified.

After discharge from the hospital, the patient was seen on the sixth postoperative day. Blake’s drain output for the patient was less than 25 mL per day since surgery and appeared seropositive. There were no signs or symptoms of postoperative infection. Blake’s bank was removed during that visit. At the patient’s six-week follow-up, the surgical incision healed well, and he returned to his usual activities and working without restriction.

Discuss

The PubMed database was used to review previously published case reports and ED case series focusing on tumor size versus symptoms and the number of cases with postoperative complications. Six individual case reports were included in our analysis, all of which reported pain at presentation with tumor size ranging from 5 x 5 cm to 13.5 x 8.7 cm (Table) 1). No complications or postoperative recurrence were reported in any of the case reports.

Published case reports, first author, general Tumor size (cm) symptoms Complications Repetition
Falidas et al., (2013) [1] 6.7 x 2.9 Pain no one number
Go et al. , (2014) [3] 10 x 6 Pain no one number
Karrakchou et al., (2017) [4] 7.7 x 2.1 (right) Pain no one number
Karrakchou et al., (2017 .) [4] 8.3 x 2.1 (left) No visible symptoms no one number
Sarishi et al (2014) [5] 5 x 5 (right) No visible symptoms no one number
Sarishi et al (2014) [5] 7 x 5 (left) Pain no one number
Pyne et al., (2002) [6] 5 x 5 Pain no one number
Güzel et al., (2020) [7] 13.5 x 8.7 Pain no one number

The postoperative complication rate among the forty-two patients represented in the three case series analyzed was twenty-six percent (Table 2). There was no clear association between tumor size and pain at presentation as there were patients who developed painful tumors of 3 cm in size, while other patients remained asymptomatic with large tumors of up to 11 cm.

Published Series, First Author, Year n Asymptomatic patients (%) Tumor diameter (cm) complication (%)
Nagano (2014) [8] 20 65 4.5 – 11 40
Tsikinis (2014) [9] 6 NR 6.0-13.0 0
Karakurt (2014) [10] 16 100 3.0-13.0 19

Dorsal fibrosarcoma (ED), initially described as rare, was first reported and named by Jarvi et al in 1961, and later classified as benign fibroma/myofibromatosis in 2000 by WHO Globalism [11]. Approximately 99% of elastic fibroadenomas are usually found in the soft tissues of the lower corners of the shoulder, in the depths of the latissimus dorsi, the anterior and rhomboid serratus, or lateral in the ribs and intercostal muscles [12]. It is traditionally considered a pseudotumor resulting from repetitive mechanical stress between the tip of the scapula and the chest wall, resulting in microtrauma and overproduction of elastic tissue from stimulating fibroblasts. [8]. However, the exact etiology remains unclear.

Since vigorous and repetitive movement of the shoulder girdle appears to be involved in causing ED, an investigation was conducted to look at the relationship between manual/heavy activities and the development of ED which showed a variable association from 15% to 95%, with male patients appearing to be more affected due to participation High in manual/heavy activities compared to females (71% and 30%, respectively) [2]. In two different retrospective studies completed, bilateral ED appeared to be more common in up to 66% of patients. [11]. A study by Haihua et al showed that risk groups include women aged 50-70 years and men aged 40-60 years. [12]. As previously mentioned, ED has traditionally been described as rare but is now thought to be more common since most small lesions remain asymptomatic with several studies showing CT evaluation and autopsy series revealing a prevalence of 1.6% to 16.5% [2].

In cases of symptomatic ED, pain is the most common major complaint, and presentation ranges from mild to severely disabling pain. It is assumed that the severity of the pain depends on the size and location of the tumor. However, based on our review, there was no clear association between tumor size and symptoms, with very small tumors presenting with pain and very large tumors remaining asymptomatic in some cases. Also, there was very little difference in tumor location from our review with the majority of tumors located in the subpolar region between the dentate and lattice muscle. There is very little information from the existing literature about the contributing factors to painful ED. Further studies should be conducted to evaluate the factors that cause ED pain. [3-9].

The treatment of ED is still controversial. Given the benign nature of the lesion, surgery is intended for symptomatic ED. The decision to remove ED should be made jointly by the patient and the surgeon. Those with significant symptoms should be offered circumcision and be aware of the risks of surgery [13]. The most common postoperative complications reported in the literature were seromas and hematomas. However, most patients had no complications after surgery. In addition, the use of a drainage tube after surgery and pressure dressings reduce the incidence of this complication. Furthermore, recurrence rates have been shown to be very low, with most patients reporting complete resolution of symptoms after surgical excision [10].

Conclusions

Further investigations are needed to identify factors that contribute to pain in patients with ED as the magnitude of ED does not appear to correlate with symptoms. Because postoperative complications and recurrence rates are low, we recommend that surgical resection be considered in all patients with painful or symptomatic erectile dysfunction.