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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 2  |  Page : 252-254
Periductal stromal sarcoma of breast with lipoblast-like cells: A case report with review of literature


Department of Pathology, Kasturba Medical College, Manipal, Karnataka - 576 104, India

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   Abstract 

The specialized mesenchyme of the breast is the seat of neoplasms with distinct morphology, though with considerable overlap due to the ever increasing number of variants. This article seeks to describe the morphologic features of one such rare neoplasm. A 35-year-old female patient presented with a breast lump that was subsequently excised. Morphological assessment and immunohistochemistry were performed and a diagnosis of periductal stromal sarcoma with lipoblast-like cells (pseudolipoblasts), was made. Being an unusual finding in a rare entity of intermediate grade, it has to be considered, before rendering a diagnosis of a malignant, higher-grade neoplasm.

Keywords: Cystosarcoma phyllodes, periductal stromal sarcoma, pseudolipoblast

How to cite this article:
Rao AC, Geetha V, Khurana A. Periductal stromal sarcoma of breast with lipoblast-like cells: A case report with review of literature. Indian J Pathol Microbiol 2008;51:252-4

How to cite this URL:
Rao AC, Geetha V, Khurana A. Periductal stromal sarcoma of breast with lipoblast-like cells: A case report with review of literature. Indian J Pathol Microbiol [serial online] 2008 [cited 2019 Nov 22];51:252-4. Available from: http://www.ijpmonline.org/text.asp?2008/51/2/252/41673



   Introduction Top


Since the original descriptions of cystosarcoma phyllodes by Chelius and Muller, [1] neoplasms arising in the breast parenchyma have been the subject of various studies and papers. Breast neoplasms of connective tissue origin have been categorized morphologicaly as cystosarcomas (fibroepithelial neoplasms), pure connective tissue tumors (without epithelial component, resembling their soft tissue counterparts) and periductal stromal tumors with intermediate features. The paucity of literature on periductal stromal tumors could be due to the fact that, earlier this rare lesion was clubbed with cystosarcoma. It was recently recognized as a separate entity and currently holds its own in the World Health Organization (WHO) classification. [2],[3],[4]


   Case History Top


A 35-year-old woman presented with a painless breast lump of 3 months duration. A clinical diagnosis of fibroadenoma was offered. The scar of previous surgery performed elsewhere was seen over the lump, details and histopathology report of which were unavailable. Subsequently, lumpectomy was performed. In a follow-up period of 4 months, patient has not shown any symptoms or signs of a recurrence.

Pathological findings

Grossly, the specimen with attached skin weighed 36 g, measured 7 × 6 × 3 cms and was a nodular, lobulated circumscribed mass with grey white areas and fatty streaks.

Microscopy showed a circumscribed tumor composed of numerous ductular units with epitheliosis, apocrine snouting, prominent myoepithelial layer surrounded by spindle cell stroma forming "ductular stromal lobules" [Figure 1]. No broad papillae or leaf-like structures were observed. The stromal cells showed extensive nuclear pleomorphism, cytoplasmic vacuolation, multinucleation, hyperchromasia, few abnormal mitoses and moderate mitotic activity (3/10 high power field (HPF)) [Figure 1] and [Figure 2]. Interlobular benign adipose tissue islands were seen infiltrated by spindle-shaped tumor cells, along with few pseudolipoblasts, which resembled malignant lipoblasts with abundant cytoplasm with macrovacuoles indenting a large nucleus [Figure 2]. Immunohistochemistry showed the tumor cells including pseudolipoblasts to be CD34 positive [Figure 3] and S100, ER, PR negative. The ductal cells were ER, PR positive, while the myoepithelial cells were S100 positive.


   Discussion Top


Earlier considered to be a variant of Cystosarcoma with adipose metaplasia [4],[5],[6],[7],[8] and not finding much mention as a distinct entity, periductal stromal sarcomas (PDSS) have since diversified into their own. The incidence is found to be higher among pre- and postmenopausal women. [4] The scant literature available discusses this tumor as a biphasic breast tumor like the cystosarcoma, but arising from the periductal stroma, unlike phyllodes which arises from the intralobular stroma. [4] These are to be distinguished from both phyllodes tumor, benign and malignant [9] and periductal stromal hyperplasia. An absence of the phyllodes architecture with no distortion of the lobular unit is the most characteristic feature. Besides this, criteria laid down by Armed forces institute of pathology (AFIP) include: (1) A predominant spindle cell proliferation of variable cellularity and atypia around open tubules and ducts devoid of a phyllodes pattern, (2) one or multiple nodules separated by adipose tissue, (3) stromal mitotic activity of three or greater in 10 high-power fields and (4) stromal infiltration into surrounding breast tissue. Periductal stromal hyperplasia has similar features, but with a lesser degree of atypicality, mitotic activity and cellularity. [4]

These tumors are grossly quite well circumscribed, nodular and often well delineated. Arising as they do from the periductal stroma, these tumors are characterized by a hypercellular proliferation of spindle cells forming cuffs around well-preserved ductal units with infiltration of the fat and surrounding tissue. Adjacent cuffs may coalesce to form large nodules and extend into lobules, surrounding open tubules and ducts. This is in contrast to mammary stromal sarcomas which displace normal mammary tissue, entrapping ducts and lobules peripherally. [4],[5] Depending on the atypia and mitotic activity, they range from being low to high grade. [4] On immunohistochemistry, these tumors are found to be CD34 positive and lack S100, ER and PR expression.

The present case exhibited all features defined by AFIP, fitting well into the PDSS category. However, the atypicality suggested a higher grade. The lipoblast-like cells did suggest a differential of a liposarcoma, arising focally in the PDSS. These cells may mislead the pathologist into interpreting this as a liposarcoma. Absence of other features of liposarcoma and immunohistochemical findings (i.e., positivity of the cells to C34 with negative S100), helped in confirming that the cells were not lipoblasts. [4]

Periductal stromal sarcomas are thought to be a tumor of intermediate behavior; resection with adequate margins is generally considered sufficient. However, its tendency to recur and the eventual progression of some of these into Phyllodes or soft tissue type sarcomas, warrants a close follow-up. This patient is currently recurrence free. Further, PDSS may occasionally exhibit intraepithelial changes ranging from ordinary hyperplasia to intraductal carcinoma.

This article presents the histopathologic features of a rare tumor, the periductal stromal sarcoma of the breast, with a hitherto unmentioned feature of lipoblast-like cells, closely mimicing Phyllodes tumor with liposarcomatous component. An awareness of this recently discovered entity along with immuno histochemistry (IHC), could avoid mislabeling these rare, biphasic tumors as Phyllodes or a soft tissue type stromal sarcoma of the breast.

 
   References Top

1.Oberman HA. Cystosarcoma Phyllodes: A clinicopathologic study of hypercellular periductal stromal neoplasm of breast. Cancer 1965:18:697-710.  Back to cited text no. 1    
2.Tavassoli FA, Devilee P, editors. World Health Organisation Classification of Tumours 2003, Pathology and Genetics Tumours of the Breast and Female Genital Organs. Lyon: IARC Press; 2003. p. 101-2.  Back to cited text no. 2    
3.Tavassoli FA. Pathology of the Breast, 2 nd ed. Nowalk, Connecticut: Appleton Lange; 1999. p. 239-60  Back to cited text no. 3    
4.Burga MB, Tavassoli FA. Periductal stromal tumour: A rare lesion with low grade sarcomatous behaviour. Am J Surg Pathol 2003;27:343-48.  Back to cited text no. 4    
5.Powell CM, Rosen PP. Adipose differentiation in cystosarcoma phyllodes: A study of 14 cases. Am J Surg Pathol 1994;18:720-7.  Back to cited text no. 5  [PUBMED]  
6.Oberman HA, Nosaanchuk JS, Finger JE, Mich AA. Periductal stromal tumours of breast with adipose metaplasia. Archiv Surg 1969;98:384-7.  Back to cited text no. 6    
7.Callery CD, Rossen PR, Kinne DW. Sarcoma of the breast - Study of 32 patients with reappraisal of classification and therapy. Ann Surg 1985;201:527-32.  Back to cited text no. 7    
8.Norris HJ, Taylor HB. Sarcoma and related tumours of the breast. Cancer 1968;22:22-8.  Back to cited text no. 8  [PUBMED]  
9.Rosai J. Breast, In Rosai J. ed. Rosai and Ackerman's surgical pathology. 9 th ed. New Delhi: Mosby; 2004. p. 1853.  Back to cited text no. 9    

Top
Correspondence Address:
Anuradha C.K Rao
Department of Pathology, Kasturba Medical College, Manipal, Karnataka - 576 104
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.41673

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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