Breast Sarcomas: Rare but Challenging Entity for Diagnosis

Background: Breast sarcomas are extremely rare, can be primary or secondary, pose a high risk of recurrence with an overall poor prognosis. Primary breast sarcomas constitute < 1% of all primary breast malignancies and less than 5% of all sarcomas. Sarcomas arising in phyllodes tumors account for < 6 % of all phyllodes tumors. The most common subtype of both primary and secondary sarcoma is angiosarcoma. Methods: This was a retrospective study of patients diagnosed as sarcoma of breast (excluding malignant phyllodes and metaplastic carcinoma) during the period from January 2016 to July 2019. The demographic and clinical features were noted from the medical records. The details of gross specimens (grossed in strict accordance with CAP protocols) were noted. The haematoxylin and eosin-stained slides along with immunohistochemistry (IHC) (pan cytokeratin, calponin, vimentin, SMA, CD117, Ki 67, desmin, CD10, CK5/6, EMA, p63, CD34, CD56, myogenin, CD99, p16, S100, BCL2, CD31, caldesmon and CD68) were reviewed and categorized according to WHO (2019) criteria. Result: The median age of patients was fifty years. There were fourteen breast sarcomas including one arising in phyllodes tumor during the study period. These included undifferentiated pleomorphic sarcoma (4), liposarcoma (2), myxofibrosarcoma (2), angiosarcoma (2), one each of leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma and stromal sarcoma. Despite using a wide panel of IHC markers, 4/14 (28.57%) sarcomas were classified as undifferentiated pleomorphic sarcoma. Conclusion: Primary breast sarcomas are rare tumors and pose diagnostic challenge. It is important to categorize these entities in view of the differing biologic behavior and molecular profiles. Morphology along with IHC is important for diagnosis, treatment and prognosis.

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Epithelial-Myoepithelial Carcinoma of the Breast with Rhabdoid Features

Case Reports in Pathology ◽

10.1155/2020/8879035 ◽

2020 ◽

Vol 2020 ◽

pp. 1-4

Author(s):

Karl Grenier ◽

Gulbeyaz Altinel ◽

Zari Dastani ◽

Atilla Omeroglu

Keyword(s):

Clear Cell ◽

Phyllodes Tumor ◽

Myoepithelial Cells ◽

Metaplastic Carcinoma ◽

Myoepithelial Carcinoma ◽

Diagnostic Challenge ◽

Carcinoma Of The Breast ◽

Epithelial Myoepithelial Carcinoma ◽

Rhabdoid Differentiation ◽

Rhabdoid Features

Epithelial-myoepithelial carcinoma of the breast is a rare biphasic tumor composed of intermixed malignant epithelial and myoepithelial components. Myoepithelial cells are known to adopt varied morphologies, including spindle, chondroid, clear cell, and rhabdoid morphologies, and can represent a diagnostic challenge when isolated on biopsy. Rhabdomyosarcoma, phyllodes tumor, metaplastic carcinoma, and myoepithelial carcinoma are primary breast tumors that all have been shown to exhibit rhabdoid features, whether representing true differentiation or morphological mimic. We here report an epithelial-myoepithelial carcinoma of the breast with rhabdoid features in a 76-year-old woman. The rhabdoid-appearing myoepithelial cells are negative for myogenin, consistent with a rhabdoid-like morphology rather than a true rhabdoid differentiation, comparably to previously described myoepithelial carcinoma with rhabdoid features. To our knowledge, this is the first reported case of epithelial-myoepithelial carcinoma of the breast with rhabdoid features and thus adds another entity to the differential diagnosis of breast lesions with rhabdoid features.

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Malignant Phyllodes Tumor with Lipomatous Differentiation: A Unique Presentation

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqaa161.074 ◽

2020 ◽

Vol 154 (Supplement_1) ◽

pp. S36-S36

Author(s):

P Johal ◽

C Sebastiano

Keyword(s):

Phyllodes Tumor ◽

Excisional Biopsy ◽

Surrounding Tissue ◽

Beta Catenin ◽

Significant Past Medical History ◽

Ki 67 ◽

Complete Excision ◽

Malignant Phyllodes Tumor ◽

Phyllodes Tumors ◽

Wide Range

Abstract Casestudy Phyllodes tumors (PT) are uncommon biphasic fibroepithelial neoplasms that account for <1% of all breast tumors. The distinction between benign and malignant PTs is made based on a number of histologic characteristics, including an infiltrative margin, stromal overgrowth, mitotic count, hypercellularity and atypia. 30% of cases show heterologous differentiation, which mostly occurs in stromal elements. This can result in a wide range of tumor histopathology including lipomatous areas. Results We present the case of a 46-year-old female with a 3.1 cm left breast palpated mass and no significant past medical history. Ultrasound guided needle biopsy revealed multiple foci of hypercellular spindle cells with marked atypia and mitoses (ki-67: 40–60%) surrounded by focal fat necrosis. Immunohistochemical staining was negative for CK 5/6, p63, CAM 5.2, SMA, desmin, AE1/3, CD163, nuclear beta-catenin and CD34. The patient subsequently underwent a wire localization excisional biopsy. Microscopic examination revealed a biphasic hypercellular lesion with both stromal and distorted glandular elements and extensive lipomatous differentiation. Although mostly well- circumscribed, some infiltration into the surrounding tissue was identified as well as nuclear atypia and an increased mitotic rate (10 mitoses/HPF). The diagnosis of malignant phyllodes tumor with lipomatous differentiation was made based on these findings. FISH was performed and was negative for MDM2, suggesting that the lipomatous component itself was not malignant (i.e. liposarcoma) Conclusion Malignant phyllodes tumors rarely show heterologous differentiation. A literature review revealed that most lipomatous differentiation was liposarcomatous with rare cases of pure lipomatous metaplasia. Although malignant phyllodes tumor have the highest rate of metastasis relative to their benign counterparts, complete excision has resulted in a mortality rate of approximately 12%. The clinical implications of lipomatous differentiation are currently unknown. However, in these cases ensuring the lack of malignant liposarcomatous tumor growth is imperative.

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Study of Anlotinib Hydrochloride and Toripalimab in Subjects With Unresectable or Metastatic Undifferentiated Pleomorphic Sarcoma

Case Medical Research ◽

10.31525/ct1-nct03946943 ◽

2019 ◽

Author(s):

Keyword(s):

Undifferentiated Pleomorphic Sarcoma ◽

Pleomorphic Sarcoma

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Undifferentiated Pleomorphic Sarcoma of Lower Extremity

Asian Pacific Journal of Health Sciences ◽

10.21276/apjhs.2017.4.2.17 ◽

2017 ◽

Vol 4 (2) ◽

pp. 102-104

Author(s):

Sunil Vitthalrao Jagtap ◽

◽

Cyrus Dara Jokhi ◽

Swati S Jagtap ◽

Ritvij Patankar ◽

...

Keyword(s):

Lower Extremity ◽

Undifferentiated Pleomorphic Sarcoma ◽

Pleomorphic Sarcoma

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Undifferentiated pleomorphic sarcoma in oropharyngeal mucosa of patients with multiple basal cell carcinomas

Rare Tumors ◽

10.1177/20363613211026483 ◽

2021 ◽

Vol 13 ◽

pp. 203636132110264

Author(s):

Andrea Dekanić ◽

Marko Velepič ◽

Margita Belušić Gobić ◽

Ita Hadžisejdić ◽

Nives Jonjić

Keyword(s):

Differential Diagnosis ◽

Basal Cell ◽

Polypoid Lesion ◽

Older Patient ◽

Mesenchymal Tumors ◽

Undifferentiated Pleomorphic Sarcoma ◽

Pleomorphic Sarcoma ◽

Basal Cell Carcinomas ◽

History Of ◽

Multiple Basal Cell Carcinomas

Malignant mesenchymal tumors of oropharyngeal mucosa are rare. Those with fibroblastic and histiocytic differentiation in the skin are called atypical fibroxanthoma (AFX) and in the soft tissue undifferentiated pleomorphic sarcoma (UPS). Here we present a case of an older patient with a history of multiple basal cell carcinomas and recently with a rapidly growing polypoid lesion in the mucosa of posterior oropharyngeal wall with AFX/UPS morphology. The differential diagnosis, histological pitfalls of this poorly characterized mesenchymal lesions, and the challenges associated with treatment are discussed.

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Undifferentiated Pleomorphic Sarcoma of Vulva

Indian Journal of Gynecologic Oncology ◽

10.1007/s40944-020-00431-6 ◽

2020 ◽

Vol 18 (3) ◽

Author(s):

Amita Naithani ◽

Parul Sharma ◽

R. Pratima ◽

Priyanka Makkar ◽

Sunil Pasricha ◽

...

Keyword(s):

Undifferentiated Pleomorphic Sarcoma ◽

Pleomorphic Sarcoma

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Splenic undifferentiated high grade pleomorphic sarcoma of a small size with fatal tumor rupture

Journal of Pathology of Nepal ◽

10.3126/jpn.v1i2.5411 ◽

1970 ◽

Vol 1 (2) ◽

pp. 151-153 ◽

Cited By ~ 3

Author(s):

BM Amatya ◽

M Sawabe ◽

T Arai ◽

T Kumakawa ◽

K Takubo ◽

...

Keyword(s):

Past History ◽

Distant Metastases ◽

Tumor Rupture ◽

Retroperitoneal Hemorrhage ◽

Undifferentiated Pleomorphic Sarcoma ◽

Bone Marrow Histology ◽

Pleomorphic Sarcoma ◽

Fatal Bleeding ◽

History Of ◽

The Right

(The order of authors on this article was changed on 09/01/2012.)Primary undifferentiated pleomorphic sarcoma of the spleen is a rare and highly aggressive neoplasm that usually presents with splenomegaly, constitutional symptoms and frequent distant metastases. We report a case of 77-year old male patient with a past history of dissecting aortic aneurysm that developed acute hemorrhagic shock. Aneurysmal rupture was clinically suspected, but the postmortem examination revealed a 25 mm-sized tumor in an atrophic spleen weighing 65 gram with massive retroperitoneal bleeding. Metastases were found in the right renal hilus, the right adrenal gland and femoral bone marrow. Histology of the tumor showed undifferentiated pleomorphic sarcoma. Tumor rupture with fatal bleeding and systemic metastases had occurred despite the small size of the tumor. Tumor size is not a reliable predictor of systemic metastasis or tumor rupture for splenic undifferentiated pleomorphic sarcoma. Keywords: Undifferentiated pleomorphic sarcoma; Malignant fibrous histiocytoma; Splenic neoplasms; Retroperitoneal hemorrhage DOI: http://dx.doi.org/10.3126/jpn.v1i2.5411 JPN 2011; 1(2): 151-153

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