Primary Primitive Neuroectodermal Tumor of the Breast: A Rare Case Presentation

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<ul><li><p>CASE REPORT</p><p>Primary Primitive Neuroectodermal Tumor of the Breast:A Rare Case Presentation</p><p>Pradyumna K. Sahoo &amp; Supti Mukhopadhyay &amp;Palash Kumar Mandal &amp; Samindra N. Basak</p><p>Received: 4 February 2011 /Accepted: 25 June 2012 /Published online: 7 July 2012# Association of Surgeons of India 2012</p><p>Abstract A primitive neuroectodermal tumor (PNET) inthe breast developed in a 36-year-old Indian woman whoinitially underwent lumpectomy and was diagnosed as themalignant phyllodes tumor of the right breast. Within2 months it recurred, clinicoradiologically appearing likeorganized collection. Incision and drainage along withbiopsy was done. The tissue diagnosis was reported asPNET. The histopathology report showed the tumorcells as malignant round cells, immunohistochemicallypositive for CD99, vimentin and neuron-specific enolase(NSE) (patchy) and negative for CD45, cytokeratin,S100, and desmin. Extended simple mastectomy wascarried out. She came after another interval with recur-rence. Chemotherapy as well as radiotherapy was given.After 18 months of surgery, the patient is having per-sistent stable disease without distant metastasis. PNETin adults is rare and has been reported in the chest wall(Askin tumor) and other visceral sites. To our knowledge,only a few cases have been reported of a primary PNET ofthe breast.</p><p>Keywords Primitive neuroectodermal tumor . Malignantphyllodes tumor . Breast</p><p>Case Report</p><p>A 36-year-old married Indian woman was referred to CancerCentre Welfare Home and Research Institute after lumpec-tomy as a malignant phyllodes tumor in the right breast.</p><p>On examination of the right breast, no mass was feltexcept a scar mark of previous lumpectomy. Mammogramand ultrasonography of both breasts did not reveal anypositive finding. USG abdomen and chest X-ray reportswere normal. The review of lumpectomy histopathologyslides in our institute reported it as same (i.e., malignantphyllodes tumor). Wide excision was done with a goodmargin; after nearly 1 month of the first surgery, histopath-ological examination (HPE) reported no residual tumor.</p><p>After 2 months, the patient presented again with the lumpmeasuring 8 cm5 cm and pain in the right breast. Clinicalimpression was serous collection with some solid compo-nent. USG breast showed multiple areas of heterogenousechotexture with multiple septae, impressing organized col-lection. Fine-needle aspiration cytology (FNAC) wasreported as duct carcinoma but it did not corroborate clini-cally. Incision and drainage was done. Blood-mixed fluidwith a few friable tissues came out. The histopathologyreport showed sheets of neoplastic round cells with highnucleocytoplasmic ratio and coarse chromatin. Brisk mitoseswere noted. A few pseudorosettes and rosettes were seen.With immunohistochemistry (IHC), the report came as PNET(vimentin positive, CD99 strongly positive, NSE patchypositivity; CD45, cytokeratin, S100, and desmin negative).</p><p>Right extended simple mastectomy with en-bloc resec-tion of pectoralis major muscle was done (Fig. 1a). On grossexamination of specimen, a grayish white fleshy soft masswith partly necrotic area was found. Final diagnosis wasgiven as PNET of the right breast based on IHC findings(Fig. 2). Level I lymph nodes were reactive. The pectoralismajor muscle was involved, but all margins were free. The</p><p>P. K. Sahoo (*) : S. N. BasakDepartment of Surgical Oncology,Cancer Centre Welfare Home and Research Institute,Mahatma Gandhi Road, Thakurpukur,Kolkata 700063, West Bengal, Indiae-mail:</p><p>S. N. Basake-mail:</p><p>S. Mukhopadhyay : P. K. MandalDepartment of Pathology,Cancer Centre Welfare Home and Research Institute,Mahatma Gandhi Road, Thakurpukur,Kolkata 700063, West Bengal, India</p><p>Indian J Surg (June 2013) 75(Suppl 1):S283S285DOI 10.1007/s12262-012-0685-3</p></li><li><p>initial lumpectomy slides were reviewed and confirmed asPNET of the breast.</p><p>The patient was discharged uneventfully with plan ofchemotherapy.</p><p>The patient did not turn for follow-up. After 4 months sheagain presented with the recurrent tumor over themedial part ofright pectoral region covering sternum (Fig. 1b and c) of 9 cm6 cm. She received six cycles of vincristine, doxorubicin, andcyclophosphamide followed by radiotherapy. There was partialresponse, so she again received three cycles of chemotherapy(ifosfamide, etoposide, and mesna). After 18 months of sur-gery, the patient is having persistent stable disease withoutdistant metastasis. Chemotherapy is still continued.</p><p>Discussion</p><p>PNET belongs to Ewings sarcoma family of tumors (ESFT)[1]. It is a small, round, blue cell sarcomatous neoplasm ofbone and soft tissue that arises from neural crest [2, 3]. Itgets its name PNET because the majority of the cells in thetumor are derived from neuroectoderm, but have notdevoped and differentiated in the way a normal neuronwould do and so the cell appears primitive [3]. PNETprimarily occurs in children and adolescents [3]. It is a raretumor in the breast [2, 4, 5].</p><p>The defining feature of ESFT includes a characteristic(11:22) chromosomal translocation and near universal ex-pression of CD99 antigen, though other translocations dooccur [1, 2].</p><p>Primary PNETs demonstrate a predilection for the truncaland axial soft tissues including the chest wall (Askin tumor),the paravertebral region (5060 % of cases), and the ex-tremities (2025 % of cases) [4]. The thoracopulmonaryregion (Askin tumor) is the single most common primarysite [4]. Primary PNET of many organs such as the kidney,ureter, bladder, testis, seminal vesicles, ovary, pancreas,uterus, parotid gland, and lungs have been documented butonly a very few case reports of primary PNET of the breastexist [4, 5].</p><p>In our case, radiation and prolonged chemotherapy wasnecessary because of initial misdiagnosis.</p><p>Since the treatment is radically different from a ductcarcinoma and malignant phyllodes tumor, we insistthat rare-appearing tumors should always be confirmedby immunohistochemistry. This will definitely help the</p><p>A</p><p>B C</p><p>Fig. 1 a Right extended simplemastectomy specimen, bRecurrent tumor, c CECTshowing the recurrent tumor</p><p>Fig. 2 Small round cells with pseudo-rosette, H/E stain high power x40;[Insetstrong CD99 membrane positivity(brown colour)]</p><p>S284 Indian J Surg (June 2013) 75(Suppl 1):S283S285</p></li><li><p>patient to achieve a long disease-free survival if theappropriate treatment with surgery and chemoradiationtherapy is given.</p><p>Acknowledgements The authors thanks to Dr S. Roy for her help inthe preparation of this manuscript.</p><p>References</p><p>1. Russell HV, Pappo AS, Nuchtern JG et al (2008) Solid tumors ofchildhood: Ewings sarcoma. In: DeVita VT, Lawrence TS,</p><p>Rosenberg SA (eds) DeVita, Hellman, and Rosenbergs Cancer:principles and practice of oncology, 8th edn. Williams &amp; Wilkins,Philadelphia, pp 20612067</p><p>2. Maxwell RW, Ghate SV, Bentley RC, Soo MS (2006) Primaryprimitive neuroectodermal tumor of the breast. J Ultrasound Med25:13311333</p><p>3. Primitive neuroectodermal tumour. Available via DIALOG. AccessedMay 30, 2010</p><p>4. Ko K, Kim EA, Lee ES, Kwon Y (2009) Primary primitive neuro-ectodermal tumor of the breast: a case report. Korean J Radiol 10(4):407410</p><p>5. Vindal A, Kakar AK (2010) Primary primitive neuroectodermaltumor of the breast. J Clin Oncol 28(27):e453e455</p><p>Indian J Surg (June 2013) 75(Suppl 1):S283S285 S285</p><p>Primary Primitive Neuroectodermal Tumor of the Breast: A Rare Case PresentationAbstractCase ReportDiscussionReferences</p></li></ul>