PubMed Search: Phyllodes tumor of the breast[TI] full text[SB] pathology. Its therapeutic management is based on wide surgery with free margins. Federal government websites often end in .gov or .mil. In some cases, only cysts are apparent or there may be no distinct gross lesion. case 'mail_failed': Periductal stromal tumor (PDST) is a rare biphasic tumor of the breast that exhibits low-grade malignancy and intermediate behavior. } In this study, we describe the case of a 48-year-old female who presented with a palpable mass in the A retrospective search of our Pathology database from 2000 to 2021 identified 6 PST, all evaluated according to the Armed Forces Institute of Pathology (AFIP) criteria. Before Would you like email updates of new search results? endobj PDSS is a distinct low-grade breast sarcoma, the appropriate diagnosis of which requires extensive tumor sampling and additional broad immunohistochemistry. They account for less than 1% of all breast neoplasms. As far as we know, only sporadic cases were reported (1) (2)(3)(4)(5)(6)(7)(8 . document.getElementById('btnstop_' + ids).style.display = 'none'; /* Introduction: Periductal Stromal Sarcoma (PSS), especially spindle and epithelioid types, is a rare subtype of the malignant fibroepithelial tumor with benign ductal elements and a sarcomatous stroma composed of spindle cells. Breast periductal stromal tumor (PDST) is a rare biphasic tumor, with both benign ductal epithelium and non-phyllodes sarcomatous stroma. Periductal stromal tumor: a rare lesion with low-grade sarcomatous behavior. Disclaimer, National Library of Medicine It is typically a large, fast growing mass that forms from the periductal stroma of the breast 13. }); Department of Pathology Stanford University School of Medicine Stanford CA 94305-5342 . histologic criteria for pss according to the afip include: predominantly sarcomatous spindle cell stromal proliferation around open ducts and tubules, lack of leaf-like growth pattern, one or more nodules that can be separated by adipose tissue, mitotic activity equal to or more than 3 in 10 hpf and infiltration into surrounding adipose tissue ( Its therapeutic management is based on wide surgery with free margins. When severe, mastitis can lead to a breast abscess (5%-11%) or septicemia. It is unclear whether this tumor belongs to the spectrum of phyllodes tumor or is a separate entity. Classification of breast lesions is an evolving process . Am J Surg Pathol 2003; 27: 343-8. The tumor is circumscribed. Periductal stromal tumor: a rare lesion with low-grade sarcomatous behavior. official website and that any information you provide is encrypted Multinucleated stromal giant cells can be seen in . loopSound(soundID); 2015 May;23(3):221-4. doi: 10.1177/1066896914567331. The epithelial component was intensely positive in all cases.)1. Additionally, PST's immunoprofile of positive KIT and CD34 stromal expression alongside the negative GATA3, p63, and SOX10 reactivity can aid the pathologist in excluding metaplastic carcinoma. 1, 31 these tumors have inherent recurrence and/or metastatic potential, which varies based on histologic grade.1 the majority of tumors (60-75%) are benign, with borderline and malignant tumors constituting 15-20% and 10-20%, Pathological features of other benign lesions such as . Methods. However, PDSS and PDSH may or may not form masses and vary in size, with a wide range (<1 cm to 24 cm). } It is unclear whether this tumor belongs to the spectrum of phyllodes tumor or is a separate entity. These rare fibroepithelial lesions display a biphasic morphology, characterized by a cellular stroma surrounding benign ducts. Periductal stromal tumors (PSTs) of the breast are considered as a rare subtype of phyllodes tumors. Fleet Management Shipping Salary, Copyright 2022 | who is your acotar mate female | Web Developed by handbook for relocating federal employees | All Rights Reserved. Periductal stromal sarcoma is an extremely rare malignant fibroepithelial tumor of the breast which is characterized by its biphasic histology with benign ductal elements and a sarcomatous stroma made of spindle cells and lacking phyllodes architecture. It has biphasic morphology with benign ducts and a sarcomatous stroma with no specific clinical or radiologic findings, which makes it difficult to diagnose (1-4).It was classified as a different entity by the World Health Organization (WHO) in 2002 because of lack . Las Vegas Raiders Media Guide, Occurrence is most common between the ages of 40 and 50, prior to menopause. After surgery, the patient received adjuvant chemotherapy. Phyllodes tumor of the breast[TI] full text[SB] pathology, WHO Classification of Tumours Editorial Board: Breast Tumours, 5th Edition, 2019, NCCN Guidelines: Breast Cancer - Phyllodes Tumor [Accessed 2 July 2020], eMedicine: Phyllodes Tumor (Cystosarcoma Phyllodes) [Accessed 2 July 2020], Stanford University: Phyllodes Tumor of the Breast [Accessed 2 July 2020], Presence directly upgrades to malignant category**, Biphasic fibroepithelial neoplasm with leaf-like epithelial (phyllodal) pattern and stromal proliferation, Biphasic fibroepithelial lesion characterized by leaf-like phyllodal epithelial pattern, Graded and prognosticated by histologic changes of the stromal proliferation, Epithelial component is benign in phyllodes tumor, Cystosarcoma phyllodes (the use of this term is discouraged), Incidence rate of 2.1 per 1 million women (, Benign phyllodes tumor most common (60 - 75%), followed by borderline (15 - 26%) and malignant (8 - 20%) tumors (, Benign phyllodes tumor occurs at a younger age compared with borderline and malignant phyllodes tumors (, Can occur rarely in pediatric age group and male, Reported to develop in ectopic breast tissue, including vulva (, Biphasic lesion with epithelial stromal interaction involved in tumorigenesis (, Loss of epithelial interaction in stromal component believed to lead to malignant progression (, Areas resembling well differentiated liposarcoma in phyllodes tumor lack, Increased incidence reported in Li-Fraumeni syndrome (, Commonly presents with firm, asymptomatic, mobile breast mass, Large tumor (up to 20 cm) can cause skin ulceration and pain (, Bloody nipple discharge uncommon and attributed to infarction and intraductal involvement (, Rare cases of hypoglycemia due to insulin-like growth factor II production (, Largely dependent on histologic diagnosis, Ultrasound and mammography do not accurately differentiate phyllodes tumor from fibroadenoma (, Ultrasound unreliable in grading of phyllodes tumor (, Prognosis correlates with histologic grade, Very low rate of local recurrence regardless of margin status (, Does not metastasize or cause mortality (, Recurrences may be benign but may also progress to borderline or malignant phylldoes tumors (, Behavior intermediate between benign and malignant phyllodes tumors (, Higher risk of local recurrence than benign phyllodes tumor, Sites include lung, pleura, bone, central nervous system, visceral organs and soft tissue, Presence of malignant heterologous elements, necrosis and tumor size correlated with metastasis (, Axillary nodal involvement by metastatic disease uncommon, Positive margin status associated with local recurrence, Tumor size is an independent risk factor for local recurrence (, 30 year old woman with painless breast mass (, 37 year old woman with multiple metastases including adrenal gland (, 44 year old woman with repeated local recurrence (, 47 year old woman with malignant phyllodes tumor showing liposarcomatous differentiation (, 70 year old woman with benign phyllodes tumor and concomitant ductal carcinoma in situ (, Narrow margins may be adequate for benign phyllodes tumor, Exact extent of clearance under debate, currently no consensus as to adequate margin width, while 10 mm is generally considered acceptable (, Efficacy of radiotherapy unclear, may improve local control but not overall survival in borderline and malignant phyllodes tumor (, Infiltrative in malignant and less commonly borderline phyllodes tumor, Whorled, bosselated cut surface in a leaf-like pattern, Skin ulceration, hemorrhage and cystic changes in large lesions (, Ulceration and hemorrhage do not indicate malignant behavior per se, Infarcted or very large benign phyllodes tumor can also show necrosis, Leaf-like (phyllodal) epithelial pattern formed by an exaggerated intracanalicular pattern, Subepithelial condensation with increased stromal cellularity adjacent to epithelium, Some regard tumors with predominant periductal stromal expansion (i.e., periductal stromal tumor) as a distinct subtype of phyllodes tumor, Graded into benign, borderline and malignant histologic grades, Cystic degeneration, hemorrhage, stromal hyalinization and myxoid change reported, Multinucleated stromal giant cells occasionally seen (, Can be found in phyllodes tumor of all histologic grades, Epithelium can be scarce in malignant phyllodes tumor with extensive stromal overgrowth, Incidental involvement by in situ and invasive carcinomas (, Myoepithelial layer is preserved but can be attenuated, Reduced epithelial stromal ratio compared with fibroadenomas, Higher nuclear atypia and cellularity in phyllodes tumor of higher grades, Large wavy and folded epithelial clusters, Occasionally, hyperplastic changes with enlarged and vesicular nuclei and small visible nucleoli may be seen, Increased atypia in dispersed cells in phyllodes tumor of higher grades (, Multinucleated tumor cells and marked stromal anaplasia reported in malignant phyllodes tumor (, Chromosome abnormalities increase with grade (. soundManager.url = 'http://www.soundviewelectronics.com/wp-content/plugins/compact-wp-audio-player/swf/soundmanager2.swf'; An extremely rare entity, the PST is actually classified as a subtype of phyllodes tumor, with specific criteria that are only found upon histological examination of the specimen. A 45-year-old woman came to our Hospital because of a palpable mass of the right breast. function show_hide(flag, ids) The therapeutic management of PSS is based on wide surgery with free margins, and adjuvant therapies are not required. Yt#},2Xb0hO[,fm p0+2O(?Q[zoy9I nQctt j0Q Unable to load your collection due to an error, Unable to load your delegates due to an error. Prior to puberty, breasts of both sexes have ducts with variable branching lined by cuboidal epithelium, no lobules and no necrosis; at puberty in females, lobules develop Influenced by estrogens, growth hormone and prolactin but not progesterone Premature thelarche: Onset prior to age 8 in girls with normal BMI in general population Barefoot International Boom Bag, the histologic features of periductal stromal sarcoma were defined as 1) a predominantly spindle cell stromal proliferation of variable cellularity and atypia around open tubules and ducts devoid of a phyllodes pattern, 2) one or more often multiple nodules separated by adipose tissue, 3) stromal mitotic activity of 3/10 high power fields, and This is a tumour of adult women, usually peri- or post-menopausal (a decade older than the median for phyllodes tumour). if (inputs[i].id.indexOf("btnplay_") == 0) { The mammographic and sonogr volume: volume, for ( var i = 0; i < recaptchaWidgets.length; i++ ) { Anderson Cancer Centre reported the incidence of each as benign (58%), borderline (12%), and malignant (30%). Its therapeutic management is based on wide surgery with free margins. Lobular carcinoma: classic pleomorphic lobular carcinoma. The 3rd edition terminology of 'periductal stromal sarcoma' is revised to 'periductal stromal tumour', akin to the widespread consensus to avoid the use of the term 'cystosarcoma' in the context of phyllodes tumours. window.setTimeout(function() { else if (flag == 'stop') { 2013 Nov;42(11):729-34. } endstream and transmitted securely. Epub 2020 Jun 9. and transmitted securely. Breast periductal stromal tumor (PDST) is a rare condition and accounts for less than 1% of all breast malignancies. Int J Surg Pathol. XyH 5{ZHjh5f(q(Z8Rl"Zp}VqJ~1q\gUt}1gWXE%.1_b0Kd!`L8t %9x!..Tb)n"%&kw#F&C.0iHWW|A^t7sk,k'P)"PzA[*e::YXZV+A~cW(:)%E^{JH?blOeX=K2=[5R:4(40"0]jRm|PpowrnMS@s\(3kS9u3y3E.auxjvqRAjj{ryP=6g=O,*G6d{B~{wZjZ"g%Sx*)\]{ var pattern = /(^|\s)g-recaptcha(\s|$)/; Accessibility Periductal stromal tumors (PSTs) of the breast are considered as a rare subtype of phyllodes tumors. Contributed by Joshua J.X. Intratumoural heterogeneity is frequently present in these tumours, making accurate histologic evaluation challenging. for ( var i = 0; i < forms.length; i++ ) { sharing sensitive information, make sure youre on a federal A diagnosis of low-grade periductal stromal sarcoma (PDSS) with myxoid features was established. The mitotic rate is greater than 3 per 10 HPF. Phyllodes Tumor of the Breast Definition Mixed epithelial and stromal/mesenchymal proliferation of breast characterized by increased stromal cellularity and characteristic broad "leaf-like" papillae inserted into cleft-like spaces Alternate/Historical Names Cystosarcoma phyllodes Diagnostic Criteria Usually large and grossly circumscribed WHO classification of tumors of the breast. soundManager.stopAll(); 1 0 obj Periductal stromal sarcoma (PSS) is an extremely rare neoplasm arising in the connective tissue of the breast, specifically from the periductal stroma. sharing sensitive information, make sure youre on a federal [8] There growth can be slow, rapid, or demonstrate alternating growth patterns. A large series from the M.D. They were previously termed cystosarcoma phyllodes, coined by Johannes Muller in 1838, before being renamed to phyllodes tumor by the World Health Organization in 2003. Shows a combination of pericanalicular and intracanalicular epithelial patterns, Relatively uniform stromal cellularity, no subepithelial condensation, Overlap in stromal cellularity and mitosis in cellular and juvenile fibroadenomas, Spindle cells arranged in long sweeping fascicles, Epithelial component often harbors in situ or invasive carcinomatous components, Malignant heterologous element can be present, Lacks marked atypia and stromal giant cells, Very low incidence relative to malignant heterologous differentiation in phyllodes tumor and metaplastic carcinoma; correlate with clinical history, Immunohistochemistry not helpful in differentiation, Involvement by low grade ductal carcinoma in situ, Small focus of osteosarcomatous differentiation. Multinucleated stromal giant cells can be seen in . Where there is extension into lobules, the lobular architecture is preserved. [1] They account for less than 1% of all breast neoplasms. periductal stromal tumor breast pathology outlinesdepartment of genetics stanford address The stromal cells were positive for CD10 (4/4), CD34 (4/4), KIT (3/4), and SMA (3/4), and negative for GATA3 (0/6), p63 (0/6), SOX10 (0/6), ER (0/4), PR (0/4), HER2 (0/4), nuclear beta-catenin (0/5), and myogenin (0/4). if ( callback && 'function' == typeof window[ callback ] ) { Which of the following features is diagnostic of malignant phyllodes tumor? Fibroadenomas are common benign neoplasms that may be treated conservatively. 3. endobj 1, 31 these tumors have inherent recurrence and/or metastatic potential, which varies based on histologic grade.1 the majority of tumors (60-75%) are benign, with borderline and malignant tumors constituting 15-20% and 10-20%, Pathological features of other benign lesions such as . Ding N, Jiang Y, Liu H, Zheng F, Zhu S, Wang M, Yang M, Kong L, Xue H, Jin Z. 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