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| Year : 2011 | Volume
: 54
| Issue : 2 | Page : 391-393 |
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| Mammary-type myofibroblastoma of soft tissue |
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Nebojsa Arsenovic1, Khorrum E Abdulla2, Kiran S Shamim2
1 Section for Skin Pathology, Department of Cellular Pathology, PathLinks Pathology Services, Lincoln County Hospital, Lincoln, United Kingdom
2 Department of Histopathology, Kings Mill Hospital, Mansfield Road, Sutton in Ashfield, United Kingdom
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| Date of Web Publication | 27-May-2011 |
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 Abstract | | |
A 40-year-old woman presented with a 1 year history of a painless, subcutaneous lump on the right buttock. Clinical examination showed an approximately 6 cm large subcutaneous mass covered by apparently normal-looking skin. No inguinal lymphadenopathy was found. The mass was excised with the clinical diagnosis of fibroma. Histologically, the lesion was consistent with mammary-type myofibroblastoma of soft tissue, a very rare, benign mesenchymal neoplasm with myofibroblastic differentiation. After surgical excision she was free of recurrence over a period of 8 months. This article also challenges the theory that suggests the origin of this tumor to be from the embryonic mammary tissue, adding another case of a site other than the milk lines. Keywords: Extramammary, mammary-type, myofibroblastoma, soft tissue
How to cite this article:
Arsenovic N, Abdulla KE, Shamim KS. Mammary-type myofibroblastoma of soft tissue. Indian J Pathol Microbiol 2011;54:391-3 |
| Introduction | |  |
Mammary-type myofibroblastoma (MFB) of soft tissue is a benign mesenchymal neoplasm identical to the breast MFB neoplasm with a reported predilection for older males. Both are circumscribed spindle cell neoplasms exhibiting myofibroblastic differentiation and a hyalinized stroma, usually with a fatty component and prominent mast cells. The former, however, occurs in extramammary sites, mainly in the inguinal/groin area. [1] This rare, soft tissue lesion was initially described in 1987 by Wargotz et al.[2] In the English literature there have been 15 such neoplasms described to date. We report a case of a 40-year-old woman with the lesion on her buttock.
| Case Report | | |
A 40-year-old woman presented to the outpatient clinic with a nontender, slow-growing, solitary, mobile lump on her right buttock. It was located in the subcutaneous tissue and had grown to a size of 6 cm over a period of 12 months. The mass was well-circumscribed, firm to elastic, quite mobile, and painless. No local/inguinal lymphadenopathy was present. After preoperative evaluation it was marginally excised with the clinical diagnosis of fibroma.
Macroscopic examination showed a firm to elastic, greyish-whitish, well-circumscribed lump measuring 5.5 × 4 × 4 cm, which was covered by the remnants of adipose tissue [[Figure 1], left]. On sectioning, the cut surface displayed solid, white, whorled tissue with no evidence of necrosis, bleeding, or degeneration [[Figure 1], right]. Grossly, the mass appeared to be a leiomyoma.  | Figure 1: Well-circumscribed lump covered by remnants of adipose tissue (left); cut surface shows solid, white, whorled tissue without necrosis, bleeding or degeneration (right)
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Microscopy revealed a well-circumscribed but unencapsulated mesenchymal proliferation composed of haphazardly arranged fascicles of spindle to oval cells set in a collagenous stroma [Figure 2]. The spindle cells had inconspicuous nuclei and poorly defined eosinophilic cytoplasm. Mitotic activity was low with no atypical figures. No cytologically atypical or multinucleated cells were featured. Occasionally, fat lobules and scattered mast cells were observed [Figure 3]. The adipocytes did not have scalloped, hyperchromatic nuclei, which are characteristic of lipoblasts. Vessels were not prominent. No degenerative/myxoid changes or necrosis were noticed. No ectopic breast tissue was discovered within the attached remnants of the surrounding adipose tissue. The neoplastic spindle cells demonstrated strong expression of desmin along with CD34 [Figure 4] and estrogen positivity. However, there was no actin, S100, or cytokeratin expression. The features had fulfilled the criteria for the diagnosis of an MFB of soft tissue. | Figure 2: Well-circumscribed, unencapsulated mesenchymal proliferation (H and E, ×40)
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 | Figure 3: Highlighted fat lobules and scattered mast cells (Giemsa, ×100)
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The patient showed no evidence of recurrence 8 months after the operation and therefore has been discharged from follow-up.
| Discussion | | |
This article describes the clinical and pathologic characteristics of a very rare, benign mesenchymal neoplasm with features identical to MFB of the breast. To date, only 15 such tumors have been published in the English literature. [1],[3],[4],[10]
MFB of soft tissue is most common in elderly males in the age group of 35-67 years (median age: 53 years). The lesions measure from 2 to 13 cm (median size: 6 cm). [3] Similarly, breast MFB is predominantly found in males between the ages of 41 and 85 years (median age: 64 years).
MFB is a well-circumscribed neoplasm consisting of slender bipolar spindle cells arranged in fascicles of various sizes and bands of hyalinized collagen that often contain prominent mast cells. [2],[5] The tumor usually has a fatty component that shows marked intralesional and interlesional variability in prominence and occasional cases show smooth muscle differentiation. With electron microscope and immunohistochemistry, the proliferating cells seem most closely related to myofibroblasts. Immunohistochemically, the MFB cells are positive for the markers desmin and variably for CD34. [3] One third of them show actin positivity. [3] Some show variable positivity for androgen and estrogen receptors. [5] These lesions are benign with no recurrence or metastasis after surgical excision.
MFB is a rare soft tissue neoplasm of uncertain histogenesis. The androgen receptor or its ligands are possibly related to the development of MFB of the breast and diagnostically useful in differentiating it from other similar spindle cell lesions. [5] Estrogen receptor may have a similar role in female patients.
The distribution of this tumor, including the breast and extramammary groin/perineal area, may prompt the speculation that it has something to do with the embryonic mammary lines, which extend from mid-axillae to medial groins.
Along these lines, accessory breast tissue may be present with or without associated pathologic breast lesions. [9] Despite this, 3 cases in the McMenamin and Fletcher series (buttock, mid-back, paratesticular), our case (buttock), and recently reported cases in the popliteal fossa, [6] head and neck regions, [7] and the vulva [8] rather question the embryonic mammary line concept.
Here is described a case of an unusual and rare soft tissue neoplasm with histologic and immunohistochemical features identical to myofibroblastoma of the breast. The lesion is usually reported in the inguinal/groin area of an elderly man. [3] This case and the previous 5 reported cases [1],[4],[6],[7],[8] indicate the equal occurrence of this neoplasm among the sexes (3 males and 3 females) and its more varied distribution in sites, including the popliteal fossa, [6] the head and neck region, [7] and the vulva. [8] Other sites include buttock, paratesticular area and groin. This article also challenges the theory that suggests the origin being from embryonic mammary tissue, adding another case of a site other than the milk lines. In a recent to-be-published paper it is suggested that the involvement of 13q14 in some cases, appears to confirm the morphologic suggested link of cellular angiofibroma with spindle cell lipoma and MFB. [10]
In conclusion, more reports are needed to evaluate the age, sex, distribution, pathogenesis, and possible behavior of this rare and unusual mesenchymal neoplasm.
| Acknowledgment | | |
We would like to thank Dr Kiran Shahpar Shamim for help in preparation of this manuscript.
| References | | |
| 1. | Maggiani F, Debiec-Rychter M, Verbeeck G, Sciot R. Extramammary myofibroblastoma is genetically related to spindle cell lipoma. Virchows Arch 2006;449:244-7. 
[PUBMED] [FULLTEXT] |
| 2. | Wargotz ES, Weiss SW, Norris HJ. Myofibroblastoma of the breast: Sixteen cases of a distinctive benign mesenchymal tumor. Am J Surg Pathol 1987;11:493-502.
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| 3. | McMenamin ME, Fletcher CD. Mammary-type Myofibroblastoma of soft tissue: A tumour closely related to spindle cell lipoma. Am J Surg Pathol 2001;25:1022-9.
[PUBMED] [FULLTEXT] |
| 4. | Mukonoweshuro P, McCormick F, Rachapalli V, Natale S, Smith ME. Paratesticular mammary-type myofibroblastoma. Histopathology 2007;50:396-7.
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| 5. | Morgan MB, Pitha JV. Myofibroblastoma of the breast revisited: an etiologic association with androgens? Hum Pathol 1998;29:347-51.
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| 6. | Scotti C, Camnasio F, Rizzo N, Fontana F, De Cobelli F, Peretti GM, et al. Mammary-type myofibroblastoma of popliteal fossa. Skeletal Radiol 2008;37:549-53.
[PUBMED] [FULLTEXT] |
| 7. | Hox V, Vander Poorten V, Delaere PR, Hermans R, Debiec-Rychter M, Sciot R. Extramammary myofibroblastoma in the head and neck region. Head Neck 2009;31:1240-4.
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| 8. | Diwadkar GB, Barber MD. Vulvar mammary-type myofibroblastoma: A case report. J Reprod Med 2009;54:404-6.
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| 9. | Rosen PP. In: Breast Pathology. Developmental abnormalities. Philadelphia: Lippincott-Raven; 1997. p. 17-21.
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| 10. | Flucke U, Han JM, van Krieken, Mentzel T. Cellular angiofibroma: Analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma. Mod Patho 2011;24:82-9.
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Correspondence Address:
Nebojsa Arsenovic
Section for Skin Pathology, Department of Cellular Pathology, PathLinks Pathology Services, Lincoln County Hospital, Lincoln LN2 5QY
United Kingdom
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.81646
[Figure 1], [Figure 2], [Figure 3], [Figure 4] |
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