Intramuscular myxoma of the paraspinal muscles: A case report and systematic review of the literature

  • Authors:
    • Saleh Rachidi
    • Amit J. Sood
    • Tihana Rumboldt
    • Terry A. Day
  • View Affiliations

  • Published online on: November 5, 2015     https://doi.org/10.3892/ol.2015.3864
  • Pages: 466-470
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Abstract

Intramuscular myxoma (IM) is a rare mesenchymal tumor of the head and neck region. The current study reports a case of a 45-year-old man who presented with a painless neck mass. Imaging showed involvement of the levator scapulae and scalene muscles. Core needle biopsy was consistent with intramuscular myxoma. Surgical excision was performed and follow-up for 30 months showed no recurrence. The present study includes a systematic review of head and neck IMs, with a summary of the clinical and demographic param­eters of all reported cases in the head and neck region. Surgery was curative in 28 of the 29 published cases, as well as in the current case (96.7%), with the lone recurrent tumor cured following re‑resection. Females constituted 57% of the cases and the mean age was 49.7±20.4 years. Although uncommon, IM should be considered in the differential diagnosis of deep neck masses, and surgical excision is the treatment of choice with a low risk of recurrence.

Introduction

In 1863, Virchow first described myxoma as a tumor anatomically resembling the umbilical cord (1). Myxomas (from the Greek word ‘muxa’ meaning mucus) are rare, benign connective tissue tumors arising from stellate mesenchymal cells (2), comprising entities such as fibromyxoma, cardiac myxoma and intramuscular myxoma (IM).

IM is an uncommon variant of the disease that typically presents between the fourth and seventh decades, with a slight female predilection (3). The majority of IMs present as slow-growing, painless masses within the thigh muscles and lower limb girdle (4,5). By contrast, IMs are rarely found in the head and neck region (4,5).

Histopathologically, the lesions are usually recognized by their paucicellularity and minimal vascularity. Similar to other myxomas, IMs consist of fibroblasts and an abundant myxoid stroma (2), primarily composed of glycosaminoglycans and fibrous structural proteins (6). However, cases of IM displaying hypercellularity and abundant vascularity have been reported, often incorrectly leading towards a diagnosis of myxoid sarcoma (7). IMs are characterized by small stellate or spindle cells without features of atypia, mitosis and necrosis (8). Tumor cells possess small, hyperchromatic nuclei and inconspicuous cytoplasm. Immunohistochemically, they generally stain positively for vimentin and cluster of differentiation 34 (9). Mutations activating Gs (α) have been suggested to show correlation with this disease process (10).

Imaging modalities, including magnetic resonance imaging (MRI), computed tomography (CT) and ultrasonography, are useful for diagnosis, but the definitive diagnosis is histopathological. IMs display low signal intensity on T1-weighted MRI images and high intensity on T2-weighted images, with peripheral or patchy enhancement following gadolinium injection (3). CT scan evaluation typically reveals a hypodense mass in comparison to adjacent musculature, without contrast enhancement (3). Consistent with CT and MRI, ultrasonography reveals a hypoechoic lesion with a partial or complete capsule (3).

Other conditions that should be considered in the differential diagnosis include aggressive angiomyxoma, myxoid neurofibroma, low-grade fibromyxoid sarcoma, myxoid liposarcoma, low grade myxofibrosarcoma, cellular myxoma, juxta-articular myxoma and nodular fasciitis (11). IMs are located entirely inside the skeletal muscle, in contrast to myxoid liposarcomas, which are intermuscular.

Histopathologically, the absence of vascularity decreases the likelihood of sarcoma, and S-100 protein negativity excludes myxoid neurofibromas and low-grade malignant peripheral nerve sheath tumors (11). Once the diagnosis of IM is confirmed through biopsy, the treatment of choice is surgical excision (5,7).

The current study reports a new case of IM involving the levator scapulae and scalene muscles, and presents a systematic review of head and neck IMs, with a summary of the clinical and demographic parameters of all reported cases in the head and neck region.

Case report

Presentation

A 45-year-old, otherwise healthy, male presented to the Medical University of South Carolina (Charleston, USA) in March 2013 with a painless mass in the posterior of the neck that had been noticed by the patient 2 months earlier. The patient exhibited no sensory impairment, numbness or weakness of the right extremities. Physical examination revealed a deep, fixed, non-tender mass, with ill-defined borders.

Diagnosis

Ultrasound imaging showed an ill-defined, hypoechoic irregularity of a deep muscle of the right posterior neck, which corresponded to a hypodense lesion within the levator scapulae muscle on a contrast CT scan (Fig. 1). There was no significant internal flow on Doppler imaging and no lymphadenopathy. MRI revealed a 2.7×2.5×1.4-cm mass within the right superficial paraspinal musculature, likely involving the levator scapulae and scalene muscles. Pathological examination of a core needle biopsy showed spindle cells with a bland appearance, in a hypovascular, myxoid stroma, confirming the diagnosis of IM (Fig. 2).

Surgical excision

Dissection was performed down to the sternocleidomastoid muscle, which was mobilized along the posterior border. The spinal accessory nerve was identified and preserved. Electromyography was used to monitor the brachial plexus and the spinal accessory nerve. Erb's point was identified and the greater auricular nerve was preserved. Level 5 dissection of the lymph nodes was undertaken, preserving cranial nerve XI, to provide access to the tumor. The mass was palpated deep to these lymph nodes, and was located within the levator scapulae muscle, extending medially to involve the posterior and middle scalene muscles. Dissection was performed around the tumor, taking an ~1-cm cuff of muscle circumferentially around the tumor. The lesion was fully resected (Fig. 3) with tumor-free borders. Following 30 months of follow-up, no further treatment was needed and the tumor did not recur.

Literature review

Methods
Pubmed search

A comprehensive literature review of the literature was performed by searching the Pubmed-National Center for Biotechnology Information database, using the keyword search ‘intramuscular AND myxoma’. The search yielded 158 studies published prior to December 2014, 15 of which were excluded, as they were in a language other than English, leaving 143 studies. Of these, 28 included cases in the head and neck region, and were included in the present literature review. The inclusion criteria encompassed all studies with IM cases of the head and neck region that were published prior to December 2014. The exclusion criteria were as follows: i) Reports published in a non-English language; and ii) cases that had been already published in another study (i.e., duplicated cases). This yielded 28 studies (1239), with 29 cases, in addition to the currently presented case. Fig. 4 outlines the case selection method.

Statistical analysis

Using Excel software (Microsoft Corporation, Redmond, WA, USA), two-tailed Student's t-test for independent samples was performed to compare the ages of the two genders. Values are reported as mean ± standard deviation (SD). P<0.05 was used to indicate a statistically significant difference.

Results

A total of 28 studies were included in this review, constituting 29 cases of IM in the head and neck region, in addition to the currently presented case (n=30; Table I).

Table I.

Summary of the head and neck intramuscular myxomas reported in the literature.

Table I.

Summary of the head and neck intramuscular myxomas reported in the literature.

Reference no.GenderAge, yearsEthnicityAnatomical locationTreatmentFU, yearsRecurrence statusSize on PE, cmSize on CT/MRI, cmSize after excision, cm
(12)F57NAParaspinal musclesSurgical excisionNANANA2NA
(13)F63NAParasipnal musclesSurgical excision1.5No recurrenceNA27×16×38NA
(14)M74NAHyoglossusSurgical excision3No recurrence88NA
(15)M51NATemporalisSurgical excision0.5No recurrence>5NA6.5×4×3
(26)F70NASCMSurgical excision5No recurrence22×1.2×1.6NA
(27)F45AsianTrapeziusSurgical excision1No recurrence2.5×34.1×2.8×4.93.5×2.5×2
(28)M52NANasal vestibule (mimetic muscle)Surgical excision1No recurrenceNA2×1.32.5
(19)F64NAParaspinal musclesSurgical excision1No recurrence1215NA
(20)M74NAMasseterSurgical excision2No recurrence2×3NANA
(21)F  2NATrapezius and paraspinal musclesSurgical excision2No recurrence4×54.1×2.8×4.9NA
(22)M22NAScalene and SCMSurgical excision4No recurrence6×47×37×4×3
(23)F43BTemporalisSurgical excision1.5No recurrence3.5×2.5NA3.5×2×2.6
(24)F  5NADeep to trapeziusSurgical excision1No recurrence44NA
(25)F60NAPosterior scapular musclesNo intervention (monitor only)1No change in size by MRIFour deep nodulesNANA
(25)M56NARight cheekSurgical excision4No recurrenceNA3NA
(26)M62NATemporalisSurgical excision1No recurrence5×4NANA
(27)M46NAOrbicularis orisSurgical excision2Recurrence in 5 months, then no recurrence3NANA
(28)F60NATongueSurgical excisionNANA2NANA
(29)F69BLevator scapulaSurgical excision1No recurrence4×34NA
(30)F43NAMasseterSurgical excision5No recurrenceNANA2×1
(31)F16NAIntermediary tendons of the digastric muscles, bilaterallySurgical excision1No recurrence6×1.5NA1.5 each
(32)M51NAPosterior neck (recurrent after previous excision)Surgical excision16No recurrenceNANANA
(33)F79NAMasseterSurgical excisionNANA1NANA
(34)F62WPosterior neckSurgical excision10No recurrenceNANA3
(35)F46NALateral neckSurgical excision2No recurrenceNANANA
(36)F42NAForeheadSurgical excision12No recurrenceNANANA
(37)M44NAGeniohyoidSurgical excision1.5No recurrence2NANA
(38)M15WCheekSurgical excision5No recurrenceNANA2×1.5
(39)M74NACheek musclesSurgical excisionNANANANANA
Present studyM45WLevator scapulae and scaleneSurgical excision1No recurrenceNA2.7×2.5×1.44

[i] F, female; M, male; W, white; B, black; NA, not available; FU, follow-up; PE, physical examination; CT, computed tomography; MRI, magnetic resonance imaging; SCM, sternocleidomastoid.

The cases consisted of 43.3% males (n=13) and 56.7% females (n=17), with an age range of 2–79 years and a mean age (mean ± SD) of 49.7±20.4 years (males, 51.2±18.2 years; females, 48.6±22.4 years; P=0.73). The most common head and neck site was the paraspinal muscles, followed by the trapezius, masseter, cheek and temporal muscles (n=3 each).

The size of the mass on physical examination was available for 17 cases, with a length range of 2–12 cm and a mean length of 4.4±2.7 cm. Of all the cases, 96.7% (29 of 30) underwent surgery as the treatment of choice, with a recurrence rate of 3.3% (n=1). One case was monitored only and no change in size was observed upon MRI at 1 year post-diagnosis. The mean follow-up time for all patients was 3.3±3.8 years.

Discussion

IM is a benign tumor that commonly affects the skeletal muscles of the thigh (4). IM of the neck paraspinal muscles is extremely rare. Although non-invasive and non-metastatic, local impingement of adjacent muscles, nerves or arteries could result in significant functional impairments.

IM could present as part of Mazbraud's syndrome, a rare disease displaying one or more IMs with fibrous dysplasia in one or more bones (40). Therefore, patients presenting with IMs should be examined for bone lesions. The number of reported cases of this syndrome in 2004 was 55 (41).

IM is the most common form of myxoma after myocardial myxoma. In addition to IM, soft-tissue myxomas include juxta-articular myxoma, superficial angiomyxoma, aggressive angiomyxoma and nerve sheath myxoma (42). The incidence of IM is ~1 per million individuals (4,43). In descending order, IMs most commonly arise in the thighs, shoulders, buttocks and upper arms. Other organs reported in the literature include the hands, face, tongue and abdominal muscles.

We recommend imaging of deep neck masses, and when surgical resection is performed, consideration of the proximity to the phrenic nerve and brachial plexus is important.

In summary, the present study reports a case of IM of the paraspinal muscles in a 45-year-old man. Following radiographic imaging with ultrasound, CT scan and MRI, a core needle biopsy confirmed the diagnosis. Surgical excision was performed and follow-up for 30 months demonstrated no recurrence. IMs should be considered in the differential diagnosis of deep neck masses. Surgical excision has shown to be curative in the vast majority of cases, with minimal recurrence rates.

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Spandidos Publications style
Rachidi S, Sood AJ, Rumboldt T and Day TA: Intramuscular myxoma of the paraspinal muscles: A case report and systematic review of the literature. Oncol Lett 11: 466-470, 2016
APA
Rachidi, S., Sood, A.J., Rumboldt, T., & Day, T.A. (2016). Intramuscular myxoma of the paraspinal muscles: A case report and systematic review of the literature. Oncology Letters, 11, 466-470. https://doi.org/10.3892/ol.2015.3864
MLA
Rachidi, S., Sood, A. J., Rumboldt, T., Day, T. A."Intramuscular myxoma of the paraspinal muscles: A case report and systematic review of the literature". Oncology Letters 11.1 (2016): 466-470.
Chicago
Rachidi, S., Sood, A. J., Rumboldt, T., Day, T. A."Intramuscular myxoma of the paraspinal muscles: A case report and systematic review of the literature". Oncology Letters 11, no. 1 (2016): 466-470. https://doi.org/10.3892/ol.2015.3864