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LETTER TO EDITOR
Year : 2017  |  Volume : 65  |  Issue : 2  |  Page : 408-410

Malignant temporal muscle myofibroblastoma with an early recurrence in a child


1 Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication10-Mar-2017

Correspondence Address:
Pankaj K Singh
Department of Neurosurgery and Gamma Knife, Room No-717, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/neuroindia.NI_1241_15

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How to cite this article:
Singh SK, Raheja AA, Jagdevan AK, Singh PK, Purkait S, Suri VA. Malignant temporal muscle myofibroblastoma with an early recurrence in a child. Neurol India 2017;65:408-10

How to cite this URL:
Singh SK, Raheja AA, Jagdevan AK, Singh PK, Purkait S, Suri VA. Malignant temporal muscle myofibroblastoma with an early recurrence in a child. Neurol India [serial online] 2017 [cited 2017 May 23];65:408-10. Available from: http://www.neurologyindia.com/text.asp?2017/65/2/408/201830


Sir,

Inflammatory myofibroblastic tumor (IMT) is a clinically and pathologically distinctive entity. IMT is an uncommon benign neoplasm with locally aggressive behavior.[1] The lesion is known by different synonyms, including inflammatory pseudo-tumor (IPT), myofibroblastoma, myofibroblastic proliferation and inflammatory fibrosarcoma, reflecting its uncertain histogenesis.[1],[2]

There are just a handful of case reports in the literature regarding temporalis muscle myofibroblastoma, along with an undefined curative treatment. The authors here report a case of IMT involving the temporalis muscle and bone, in which patient was operated twice within a span of three months due to an early recurrence. This was followed by radiotherapy in the postoperative period making the child tumor free without any psychological or physical retardation.

The index case is a 30-month old male child, who presented with a swelling over right temporal region since the age of 1 year, that was gradually increasing in size without any neurological deficits. On examination, a 3 × 2 × 2 cm swelling was present over the right temporal region. It was mobile in nature and firm in consistency. The imaging [Figure 1]a and [Figure 1]b was suggestive of a soft tissue thickening involving the overlying muscle without any intracranial extension or bony involvement.
Figure 1: (a) Axial section, CECT head showing a right extracalvarial mass with multiple punctate densities; (b) Coronal image showing a mass at the extratemporal region; (c) Postoperative axial section after the first surgery showing total resection of the mass; and, (d) Axial scan 6 months after the second operation and radiotherapy showing no recurrence and a deficient temporal bone

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The patient was operated utilizing a right temporal linear incision. A firm-to-hard fibrous tumor, mildly vascular in nature, was resected along with 1 cm cuff of the normal temporalis muscle. Postoperatively, the patient was discharged with normal imaging without any residual tumor [Figure 1]c. On histopathological examination, the lesion was composed of diffuse storiform proliferation of spindle shaped fibroblasts and myofibroblasts in a predominantly fibromyxoid stroma interspersed with dense bands of collagen. This was accompanied by inflammatory cells which included lymphocytes, plasma cells, and foamy histiocytes. Mitotic figures were rare, but the cellularity of the tumor together with hyperchromatism, variations in the size of nuclei and the invasive quality of the tumor, indicated malignancy. Immunohistochemical analysis [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d,[Figure 2]e revealed that the spindle shaped cells arranged in short fascicles with intervening collagenous stroma (hematoxylin and eosin × 100) were strongly and diffusely positive for vimentin, smooth muscle actin (SMA), anaplastic lymphoma kinase (ALK), and revealed a high proliferative index (Ki-67 = 60%). It was immunonegative for S100 and desmin, based on which a diagnosis of myofibroblastoma with malignant transformation was established. There was no clinical evidence of distant metastatic spread.
Figure 2: (a) Photomicrograph showing spindle tumor cells arranged in short fascicles with intervening collagenous stroma (hematoxylin and eosin, ×100); (b) Photomicrograph showing plump spindle shaped cells separated by bands of collagen; (c) The tumor cells are immunopositive for SMA; (d) The tumor cells are immunonegative for S100; and, (e) The tumor cells are immunonegative for desmin

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The tumor recurred within 3 months at the same site with temporal bone involvement. It was completely resected again with excision of the involved bone. Radiotherapy was given after the second surgery. Biopsy after the second surgery also revealed the same immunohistochemistry. The patient was followed up after radiotherapy at 6 monthly intervals. There was no recurrence [Figure 1]d until the last follow up visit.

The World Health Organization (WHO) defined IMT/myofibroblastoma as an intermediate soft tissue tumor that is composed of spindle cells with myofibroblastic differentiation accompanied by numerous inflammatory cells, plasma cells and/or lymphocytes.[1] IMTs are most commonly found in the lungs, abdomen, retroperitoneum, and extremities; but their occurrence in the head and neck region is less common,[3] which includes the orbit followed by larynx, tonsils, parapharyngeal space, nasal cavity and maxillary sinus.[2],[3],[4] Whether IMT is a neoplastic or a reactive process is controversial but findings of specific genetic alteration suggests more of a neoplastic etiology than a reactive inflammatory process.[5]

Myofibroblastoma/IMT occurs more frequently in children and young adults [6] with equal incidence in male and female subjects.[4] Temporalis muscle IMT with secondary bony invasion is difficult to diagnose, since it has a large array of differential diagnostic possibilities, such as malignant otitis externa, necrotizing bacterial osteomyelitis, fungal osteomyelitis, cholesteatoma, other primary neoplasias and metastases. Radiological imaging is of paramount importance to outline the lesion, because of the latter's capacity to destroy the temporal bone and cause an intracranial invasion.[1],[5] In 2003, Williamsom et al.,[7] published a review of 10 cases of IMT with secondary bony involvement. In nine of them, they performed total surgical removal and, in one case, it was a subtotal removal. Two cases received steroids, one with a residual tumor and the another because of meningeal involvement. In the case with residual tumor, they also used radiotherapy. In 2008, Montoya et al.,[8] published a case of a 75-year old man with a temporal bone IMT, with skull base invasion and involvement of cranial nerves VI, X, XI and XII. Tympano–mastoidectomy was done in this patient. It was also having dura mater erosion and posterior cranial fossa invasion. The patient expired afterwards.

In our patient, the tumor was completely excised twice before subjecting the patient to radiotherapy. Our patient was unique due to very infrequent incidence of the tumor and due to its recurrence within 3 months after the first surgical intervention with secondary bony involvement. There is no consensus regarding the best treatment for a temporalis muscle myofibroblastoma with invasive recurrences.

On recurrence, a wide local excision followed by radiotherapy is recommended due to the highly aggressive nature of the tumor. However radiotherapy is controversial in children, especially in those less than 3 years of age due to growth related and hematological side effects along with cognitive impairment. It also often fails to provide survival advantage.[7] [Table 1] compares the authors' case with the other reported cases.
Table 1: Characteristic of cases of inflammatory pseudotumours[9]

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Temporalis muscle myofibroblastoma with secondary bony invasion is rare and represents a very distinct clinical and pathological entity. Its diagnosis should be supplemented with histopathological and immunohistochemical studies. Surgical treatment should be early and more radical due to its highly aggressive nature and osteodestructive properties. This should be followed by comprehensive radiotherapy or chemotherapy.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.[9]

 
  References Top

1.
Coffin CM, Hornick JL, Fletcher CD. Inflammatory myofibroblastic tumor: Comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol 2007;31:509-20.  Back to cited text no. 1
    
2.
Fisher C. Myofibroblastic malignancies. Adv Anat Pathol 2004;11:190-201.  Back to cited text no. 2
    
3.
Al-Sindi K, Al-Shehabi MH, Al-Khalifa SA. Inflammatory myofibroblastic tumor of paranasal sinuses. Saudi Med J 2007;28:623-7.  Back to cited text no. 3
    
4.
Ushio M, Takeuchi N, Kikuchi S, Kaga K. Inflammatory pseudotumor of the paranasal sinuses: A case report. Auris Nasus Larynx 2007;34:533-6.  Back to cited text no. 4
    
5.
Ribeiro AC, Joshi VM, Funkhouser WK, Mukherji SK. Inflammatory myofibroblastic tumor involving the pterygopalatine fossa. Am J Neuroradiol 2001;22:518-20.  Back to cited text no. 5
    
6.
Cho KJ, Lee DH, Jung SH, Kim JH. A case of an inflammatory myofibroblastic tumor of the mastoid presenting with chronic suppurative otitis media. Auris Nasus Larynx 2007;34:523-6.  Back to cited text no. 6
    
7.
Williamson RA, Paueksakon P, Coker NJ. Inflammatory pseudotumor of the temporal bone. Otol Neurotol. 2003;24:818-22.  Back to cited text no. 7
    
8.
Santaolalla-Montoya F, Ereño C, Zabala A, Carrasco A, Martínez-Ibargüen A, Sánchez-Fernández JM. Inflammatory myofibroblastic tumor of the temporal bone: A histologically nonmalignant lesion with fatal outcome. Skull Base 2008;18:339-43.  Back to cited text no. 8
    
9.
Williamson RA, Paueksakon P, Coker NJ. Inflammatory pseudotumor of the temporal bone. Otol Neurotol 2003;24:818-22.  Back to cited text no. 9
    


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