International Journal of Oral & Maxillofacial Pathology. 2012;3(1):51-54 ISSN 2231 – 2250
An Atypical Myofibroblastic Tumor of the Nasal Cavity: A Case Report
Laurence Shapiro, Ines Velez, Michael A. Siegel
Myofibroblastic tumors are difficult lesions to diagnose and treat appropriately. The clinical, radiographic and histopathologic manifestations of the disease, usually suggest more aggressive entities. In numerous cases of myofibroblastic tumors, the microscopic interpretation is of a high grade malignant tumor while the clinical behavior may be that of a benign lesion. They may range from completely benign looking to atypical, pseudo-sarcoma or sarcomatous tumors. The malignant potential is also variable with benign, locally invasive and persistent recurrences. Rarely, metastases are encountered. The head and neck surgeon must be aware of the variable nature of these tumors. This diagnosis should be considered when inflammatory disease or other benign or malignant neoplasms are excluded. The purpose of this paper is to remind the health care professional that although these lesions often appear aggressive, in numerous cases the behavior is that of an indolent benign condition. It will prevent over-treatment resulting in unnecessarily radical and potentially mutilating surgery. Cases like this, atypical myofibroblastic tumor of the nasal cavity while rarely reported, suggest that early intervention with myofibroblastic tumors and surgical excision with clear tumor margins is curative.
Keywords: Atypical;Myofibroblast;Neoplasms;High Grade;Fibromatosis;Aggressive;Head and
Laurence Shapiro, Ines Velez, Michael A. Siegel. An Atypical Myofibroblastic Tumor of the Nasal Cavity: A Case Report. International Journal of Oral & Maxillofacial Pathology; 2012:3(1):51-54. ©International Journal of Oral and Maxillofacial Pathology. Published by Publishing Division, Celesta Software Private Limited. All Rights Reserved.
Received on: 27/07/2011 Accepted on: 26/02/2012
Many tumors and tumor-like conditions contain myofibroblasts or cells that morphologically resemble them. These lesions are histopathologically categorized as benign, intermediate or malignant. Inflammatory myofibroblastic tumors which are in the intermediate category are considered to be rare in the head and neck region. To date, fewer than 30 cases have
been described in the literature.1 This is the case of a 21 year old Caucasian female with
no significant medical history. There were no physical, clinical, and radiographic or laboratory signs of this disease other than the induced discomfort when touching the ala of the nose. The purpose of this paper is
to remind the health care professional that,
although often, clinical signs of myofibroblastic tumors may appear aggressive, in numerous cases the behavior and clinical presentation are those of an indolent benign condition.
The patient is a 21-year-old Caucasian female with no significant medical history and with no reported history of tobacco or recreational drug abuse. There is no family history of cancer or associated risk factors.
The presenting symptom was mild, induced discomfort experienced by the patient when touching her nose. Other than the discomfort noted when contacting the nasal ala, there was no signs of facial deformity, swelling, asymmetry, epistaxis, discharge or associated radiographic findings.
An Ear Nose and Throat specialist made a presumptive clinical diagnosis of an infected hair follicle for which a nasal spray and topical medication were prescribed. A second opinion was similar to the initial diagnosis with the additional caveat that if the symptoms continued unabated for an additional two weeks, a biopsy should be performed. The lesion was subsequently biopsied. The gross description of the tissue from the left nasal septum consisted of a tannish fragment of soft tissue measuring
0.5 cm in greatest dimension. The histopathology disclosed an atypical myofibroblastic tumor of uncertain malignant potential. The lesion was immunoreactive for smooth muscle actin, but non-reactive for S-
100 protein. Margins of this section remained positive for tumor. The initial histopathologic diagnosis was, “mesenchymal tumor of uncertain malignant potential.”
©2012 International Journal of Oral and Maxillofacial Pathology. Published by Publishing Division, Celesta Software Private Limited . All Rights Reserved
52 Shapiro L, et al. ISSN 2231 - 2250
Further histopathological review revealed a non-encapsulated tumor, causing epithelial ulceration and composed of cells that varied from spindle to epitheliod shapes. The cells were organized in fascicles and whorls with a fibrous hyalinized and highly collagenized stroma (Fig 1). The tumor cells showed pale multivacuolated lipoblast-like cytoplasm with fusiform or round nuclei. Some hyperchromatic cells and mitotic figures were noted, as well (Fig 2). Most significantly perineural growth of the tumor was identified. There were no signs of a significant inflammatory infiltrate (Fig 3). Immunohistochemical stains were repeated and showed positivity for vimentin, desmin and smooth muscle actin. The biopsied tissue was also negative for S-100 and CD-
34. This lesion was consistent with a,
“malignant myofibroblastic tumor."
The patient was subsequently seen by the Head and Neck Service of the Memorial Sloan Kettering Hospital for Cancer and Allied Diseases. Their recommendation was for total surgical excision of the tumor. The resective surgery was performed and was immediately followed by reconstructive plastic surgery of the nasal septum. Normal post-operative healing took place without sequelae. The surgical pathology report yielded a diagnosis of sarcoma. The attending surgical and oncology teams determined that there was no need for radiation or chemotherapy based on the clear tumor margins of the resection. However, because of the non-specific nature of the histopathology, a tumor board was convened and reported a final diagnosis of, “atypical myofibroblastic tumor, low grade, of uncertain malignant potential”. This case has been followed for a ten year period without recurrent disease or additional therapy.
There are several controversial issues concerning myofibroblastic proliferations, such as whether these lesions are homogeneous entities, are true neoplasms,
and if so, their degree of malignancy.2 They have an unknown etiopathogenesis.3 Some myofibroblastic lesions may represent a reparative reaction. However, some of them are true neoplasms. The nomenclature of these lesions, as reported in the literature, is
inconsistent and confusing. Review of the
literature suggests several related mesenchymal tumors such as spindle cell tumor, nodular fasciitis, myofibroblastoma, inflammatory pseudo tumor,
myofibrohistiocytic proliferation and plasma cell granuloma. According to Enzinger and Weis the term "myofibroblastic tumor” is
preferred.4,5 It is suggested by some authors that these tumors do not represent a single clinical and/or histopathological entity but rather should be considered a generic term applied to any non-specific chronic inflammatory expanding lesion.4,6
Figure 1: Low power H & E sections showing fascicles and whorls of cells within a collagenized fibrous stroma.
Figure 2: Pale multivacuolated lipoblast-like cells.
Figure 3: Perineural growth of tumor cells.
ISSN 2231 – 2250 Atypical Myofibroblastic Tumor..... 53
Inflammatory myofibroblastic tumors in the head and neck region are considered to be rare. In the majority of cases they behave as a benign condition, but invasive, locally recurrent and metastasizing forms have also been reported. Inflammatory myofibroblastic tumor has been regarded as a response to an acute infection, as a post inflammatory reparative process and as a low grade
malignancy of spindle cells.4 These lesions may be an unusual response to trauma and localized infection. Clinical and radiologic presentation are often interpreted as malignant.3,5 Diagnosis of inflammatory
myofibroblastic tumor cannot be made without histopathologic and immunoreactant
Myofibroblastic tumors have also been described as the stromal response to a variety of benign and malignant neoplasms composed at least, in part, of myofibroblasts. Furthermore, some pathologists have classified these tumors according to their origin. Classification of tumor origin include idiopathathic, regenerative/post traumatic, developmental (embryological), functional
(endocrine), iatrogenic and infectious.6
Although these lesions are most commonly found in the lungs, multiple reports described them in virtually every anatomic location including the breast, mediastinum, gastrointestinal tract, pancreas, oral cavity,
nerve, bone and central nervous system.4
Though the age range is broad, extra- pulmonary lesions show a predilection for children and young adults, with a mean age of approximately 10 years. Females are affected at a slightly higher rate than males. Coffin, et al, in a series of 53 cases with follow up, reported no instance of
metastases.7 Weis and Enzinger reported metastases to lung and brain in 3 of 27
Benign myofibroblastic tumors, such as nodular fasciitis, are fast growing conditions, with aggressive clinical course, but are cured with local, even incomplete excision. Metastasis never occurs. Clinically intermediate myofibroblastic tumors may have local recurrences and rarely metastasize. It may include inflammatory myofibroblastic tumor and atypical myofibroblastic tumor. Malignant myofibroblastic tumors commonly presenting
recurrences and metastasis are sarcomas.4
The treatment of choice, depending on the
behavioral classification, is surgical excision with clear margins with re-excision of recurrent tumors. The benefit of radiation or
chemotherapy remains unproven.6 Some authors have used corticosteroids to treat these lesions. High doses of corticosteroids often yield a favorable response especially in early lesions that include lymphoid follicles which tend to be more responsive to
glucocorticoids. Mature lesions with more
fibrous content are less responsive to steroid therapy.7-9 Cellularity, mitotic figure counts and the extent of the inflammatory infiltrate do not appear to be prognostic indicators; in
contrast, cytologic atypia, ganglion-like cells, p53 expression and DNA aneuploidy may be
useful in identifying those tumors that are more likely to pursue a more aggressive clinical course.4,8
Myofibroblastic tumors are difficult lesions to diagnose and treat appropriately.10,11 The clinical, radiographic and histopathologic
manifestations of the disease, such as this case, may suggest more aggressive entities. In numerous cases of myofibroblastic tumors, the microscopic interpretation is of a high grade malignant tumor while the clinical behavior may be that of a benign lesion.
Atypical myofibroblastic tumors may range from completely benign looking to atypical, pseudo-sarcoma or sarcomatous tumors. The malignant potential is also variable with benign, locally invasive, persistent recurrences. Rarely, metastases are encountered. The head and neck surgeon must be aware of the variable nature of these tumors. This diagnosis should be considered when inflammatory disease or other benign or malignant neoplasms are
This case is illustrative of a benign atypical myoblastic tumor in that its clinical presentation was indolent, being absent of clinical and radiographic features. Diagnosis was initially inaccurate because of the benign, atypical nature and rarity of this tumor and was ultimately established via a biopsy when a second opinion was sought by the patient. Head and neck surgeons should be familiar with the clinical and histopathological features of myofibroblastic tumors to prevent over-treatment resulting in unnecessarily radical and potentially mutilating surgery. Cases like these, while rarely reported, suggest that early intervention with myofibroblastic tumors and
54 Shapiro L, et al. ISSN 2231 - 2250
surgical excision with clear tumor margins is curative.
1. Dr.Laurence Shapiro, DDS, Assistant Professor, Department of Cariology and Restorative Dentistry, 2. Dr.Ines Velez, DDS, MS, Professor, Department of Diagnostic Sciences, College of Dental Medicine, 3. Dr.Michael A. Siegel DDS, MS, FDS RCSEd, Professor and Chair, Department of Diagnostic Sciences, College of Dental Medicine, Department of Internal Medicine (Dermatology), College of Osteopathic Medicine, Nova Southeastern University, 3200 South University Drive, Fort Lauderdale, Florida 33328.
We would like to thank Dr.Thomas Ward, Dr.Morton Lieberman and Dr.Lina Mejia for their support and cooperation.
1. Lai V, Wong Y, Lam W, Tsiui W, Luk S.
Inflammatory myofibroblastic tumor of
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3. Soysal V, Yigitbasi O, Kahya HA, Gurney E. Inflammatory myofibroblastic tumors of the nasal cavity; a case report and review of the literature. Int J Pedi Otorhinolaryngol 2001;61:161-5.
4. Mindell, ER. Enzinger and Weiss’s Soft Tissue Tumors. 4th ed. St. Louis: Mosby; 2001.
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10. Fang JC,Dym H. Myofibroblastic tumor of the oral cavity. A rare clinical entity. NY State Dent J Ma 2004;70(3):28-30.
11. Wick MR, Humphrey PA, Ritter JH.
Pathology of Pseudoneoplastic Lesion. Philadelphia: Lippincott Williams &
12. Huang WH, Dai YC. Inflammatory Pseudo tumor of the nasal cavity. Am J Otolaryngol 2006;27(4):275-7.
13. Kempson RL, Fletcher CD, Evans HL, Hendrickson MR, Sibley RK. Atlas of Tumor Pathology. Tumors of the soft tissues. 3rd Series. Washington DC:
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Corresponding Author Dr. Laurence Shapiro, Assistant Professor,
Dept. of Cariology and Restorative Dentistry, College of Dental Medicine,
Nova Southeastern University,
3200 South University Drive, Fort Lauderdale, Florida 33328. Phone: 954-262-1909
Source of Support: Nil, Conflict of Interest: None Declared.
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