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Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome, is a multisystem antineutrophil cytoplasmic antibody (ANCA) positive vasculitis, characterized by the presence of chronic rhinosinusitis, asthma and prominent peripheral blood eosinophilia. Although the most commonly involved organ is the lung, followed by the skin, EGPA can affect any organ system. Herein, we present the complicated case of an 18-year-old male patient with severe life-threatening EGPA, with central nervous system, cardiac and gasterointestinal involvement, which was resistant to initial treatment with glucocorticoids and cyclophosphamide. The patient responded well, achieving complete remission after the addition of rituximab and mepolizumab to glucocorticoids and cyclophosphamide.

Breast carcinosarcoma is a rare malignancy accounting for approximately 0.08–0.2% of all breast tumours. It is a type of metaplastic mammary carcinoma displaying biphasic differentiation with epithelial and mesenchymal cellular characteristics with probable derivation from myoepithelial cells. A 65-year-old woman presented to our hospital with a two-month history of a rapidly growing mass in her left breast. Neither her medical nor family history was positive for malignancies. Subsequently, she underwent a fine needle aspiration, which was positive for adenocarcinoma, followed by a lumpectomy with axillary lymph node dissection. The histology revealed an undifferentiated neoplasm and the immunohistochemical cell staining was positive for pan-cytokeratin, SMA, Vimentin, and S-100, suggesting the diagnosis of breast carcinosarcoma with a triple-negative immunophenotype. There was no evidence of metastasis except for a positive lymph node, indicating a stage IIIA disease. Thus, she received adjuvant chemo-radiotherapy. However, 16 months later, she presented with metastatic disease, and unfortunately, she died 4 months later due to a systemic infection. Aggressive behavior, higher staging, chemoresistance, and higher proportion of triple negative breast cancer seem to be the main characteristics of breast carcinosarcoma, which is recognised as a separate entity from common invasive ductal carcinoma, with significantly worse clinical outcomes.

Introduction: Breast sarcomas (BS) are uncommon and often present both diagnostic and therapeutic challenges. Historically, radical surgery has been the mainstay of treatment for localized breast sarcomas. On the other hand, in advanced disease, since they are a heterogeneous group of neoplasms consisting of several different subtypes including angiosarcoma, phyllodes tumor, and pleomorphic undifferentiated sarcoma, there is a lack of proven specific therapy. As a result, their treatment is based on the soft tissue sarcoma (STS) paradigm, whereas histotype-tailored approaches apply to specific subtypes like dermatofibrosarcoma protuberans. To date, advanced stages constitute an incurable form of disease and chemotherapy remains the cornerstone of treatment with the aim of palliation of symptoms and increase in survival.Areas covered: In this manuscript, we review the clinicopathologic characteristics of the most common subtypes of BS, as well as the current treatment landscape of BS, with a particular focus on opportunities and challenges provided by new targeted molecules and immunotherapy.Expert opinion: The treatment approach of advanced BS is based on the pathologic subtype. A true breakthrough has still to be obtained, as the development of new agents in BS suffers from the same weaknesses as in other STS.

The objective of the present systematic review was the determination of methodologies preferable for treating phyllodes tumors (PTs) of the breast and whether the malignancy of the tumor is of significance to the selected treatment. In addition, to investigate if local recurrence (LR) within patients is different based on the therapeutic approach followed by the physician. All studies were gathered by utilization of the biggest online medical databases in the world including PubMed, Cochrane, Embase, Web of Science and Google Scholar. Due to the specificity of the study, the resultant set of studies included in the present analysis was not large. All included studies had to refer to patients diagnosed with PTs of the breast, include the malignancy of the tumor and the preferred treatment. Moreover, they included a reference to LR post-treatment, even if there wasn't any. The age range of patients was 20-55 years old and follow-ups should have been performed. As a result, from the initial 484 studies gathered and after proper and thorough evaluation, only 10 were of significance. The studies appeared heterogeneous in terms of population, topology, treatment methodology, additional therapeutic approaches, LR rate, age and follow-up periods. Overall, excisions were used for non-malignant tumors while mastectomy was preferable for tumors with malignancy. Radiotherapy was used both as an additional treatment for tumors and LR. Also, it was revealed that LR varied based on the malignancy and treatment methodology.

Background/Aim: Utilizing an experimental animal model, we investigated the correlation between aromatase inhibitors (AIs) (anastrozole and letrozole) and Calprotectin levels. AIs have demonstrated superior efficacy when used as adjuvant endocrine therapy or monotherapy for postmenopausal patients with hormone receptor (HR)-positive early-stage breast cancer, although various side effects have been recorded. Materials and Methods: Fifty-five adult female Wistar rats were randomized and assigned into four groups. The control group received no intervention. The other three groups were subjected to ovariectomy, and serum Calprotectin levels were measured at baseline, 2, and 4 months. In addition, glucose, total cholesterol, very low-density lipoprotein- (VLDL-) cholesterol, low-density lipoprotein (LDL-) cholesterol, high-density lipoprotein- (HDL-) cholesterol, and triglyceride levels were measured. Histological analysis of liver tissue was carried out following rats’ euthanasia. Results: Aromatase inhibitors (anastrozole and letrozole) affect calprotectin levels in ovariectomized rats. Calprotectin, a marker of inflammation, was found to be affected by the use of the inhibitors. Conclusion: The potential of hepatotoxicity can be examined by assessing the elevation of inflammation markers such as Calprotectin, which is an indicator that should be strictly taken into consideration when administering aromatase inhibitors as treatment.

The objective of this study is the investigation of the potential value of a logistic regression model for the classification of gastric cytological data. The model was based on the morphological features of cell nuclei. The aim was the discrimination of benign from malignant nuclei and subsequently patients. Cytological images of gastric smears were analyzed by an image analysis system capable to extract cell nuclear features. Measurements from 50% of the patients were selected as a training set for model creation, while the measurements from the remaining patients were used as test set to validate the results. Furthermore, a model for the classification of individual patients, based on the classification of their cell nuclei has been developed. This approach set gave a correct classification at the level of 90% on the training and test sets on the nuclear level. Concluding the application of morphometric feature selection in combination with logistic regression may offer useful and complementary information about the potential of malignancy of gastric nuclei and patient cases.

BACKGROUND/AIM Primary sarcomas of the lung (PSL) represent a rare, largely unknown entity. We herein present a retrospective study of 26 patients diagnosed with PSL. PATIENTS AND METHODS For a period of 10 years, the records of patients from 5 centers were gathered and analyzed. RESULTS Median age at diagnosis was 61.96 years (range=31-75 years). Eight patients (33.33%) had mediastinal node invasion (MNI). From 17 patients (70.83%) with localized disease, 11 patients (64.70%) underwent surgery. Recurrence rate was 72.72%. Median disease-free interval was 15 months. The median overall survival (OS) of patients with metastatic disease was 4 months and 10 months for the whole population. Only surgery had an impact on survival. CONCLUSION Prognosis of PSL is somber. The high proportion of patients with MNI at diagnosis may serve as an indication for surgical evaluation of mediastinum and raises the question whether patients with locoregional PSL should be treated with a more aggressive approach.

Objective of this study is to investigate the potential of an artificial intelligence (AI) technique, based on competitive learning, for the discrimination of benign from malignant endometrial nuclei and lesions. For this purpose, 416 liquid-based cytological smears with histological confirmation were collected, each smear corresponded to one patient. From each smear was extracted nuclear morphometric features by the application of an image analysis system. Subsequently nuclei measurement from 50% of the cases were used to train the AI system to classify each individual nucleus as benign or malignant. The remaining measurement, from the unused 50% of the cases, were used for AI system performance evaluation. Based on the results of nucleus classification the patients were discriminated as having benign or malignant disease by a secondary subsystem specifically trained for this purpose. Based on the results it was conclude that AI based computerized systems have the potential for the classification of both endometrial nuclei and lesions.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the digestive tract, followed by schwannomas, lipomas, leiomyomas, and vascular tumors. They arise more often in the stomach, followed by the small bowel, esophagus, and rectum. Imatinib mesylate, a tyrosine kinase inhibitor with activity against ABL, BCR-ABL, platelet-derived growth factor receptor-alpha (PDGFRA), and c-KIT (CD117), constitutes the cornerstone of treatment for inoperable or metastatic GIST. Cases showing disease progression or resistance to imatinib mesylate may retain their morphology or present unusual morphologic and immunohistochemical characteristics. We herein describe a case of a 67-year-old patient with a previous history of GIST of the stomach, with local recurrence, who was admitted with a workup of lung nodule on chest computed tomography as part of the routine follow-up. The nodule was resected which showed a malignant tumor composedof epithelioid cells, with an abrupt transition to chondrosarcoma. Epithelioid cells were immunostained for CD117, DOG1, and Vimentin, whereas chondrosarcomatous cells expressed only Vimentin. These findings were consistent with metachronous pulmonary metastasis of the previously diagnosed GIST with chondrosarcomatous dedifferentiation. No KIT or PDGFRA mutation was detected. A review of all accessible pertinent papers disclosed 26 similar cases with unusual morphological and immunohistochemical findings, either post-imatinib treatment or, less commonly, de novo, with heterogeneous differentiation. Awareness of the histological and immunohistochemical changes in GISTs post imatinib therapy is essential to avoid a severe diagnostic pitfall.

Metastatic involvement of the thyroid occurs rarely, by either hematogenous spread or direct extension from adjacent organs. The most frequent metastatic tumors are clear cell, renal cell, lung, breast, and squamous cell carcinoma. The occurrence of osteosarcoma and papillary thyroid carcinoma in the same patient is rare, with only a few reported cases in the literature. On the other hand, only one case of osteosarcoma thyroid metastasis has so far been reported. We herewith present another case with metastatic osteosarcoma and multifocal papillary thyroid carcinoma presenting as a collision tumor and review the relevant literature.

Background: Angiogenesis is a hallmark of breast cancer (BC) and is mediated by the vascular endothelial growth factor (VEGF) signaling axis. It is regulated by different proangiogenic factors, including platelet-derived growth factor-CC (PDGF-CC) and heparin-binding EGF-like growth factor (HB-EGF), as well as co-receptors, such as neuropilin-1, which could have prognostic implications in BC patients. Patients and methods: We assessed the serum levels of VEGF, HB-EGF, PDGF-CC and neuropilin-1 in 205 patients with early BC (invasive, n = 187; in situ, n = 18) and in 31 healthy donors (HD) and investigated the potential associations with clinical and histopathological parameters. Results: VEGF serum levels were significantly higher in patients with invasive versus ductal carcinomas in situ. PDGF-CC serum concentrations varied among BC molecular subtypes. Furthermore, we observed a differential expression of most biomarkers between overweight/obese (body mass index (BMI) ≥ 25 kg/m2) and non-obese patients among the BC molecular subtypes. Finally, the classification of subjects according to menopausal status revealed a significant difference in specific biomarker levels between patients and HD. Conclusion: The serum concentrations of angiogenic molecules differ among breast cancer molecular subtypes and are affected by the BMI and menopausal status, which could have possible clinical or prognostic implications.

Objective: This study investigates the potential of an artificial intelligence (AI) methodology, the radial basis function (RBF) artificial neural network (ANN), in the evaluation of thyroid lesions. Study Design. The study was performed on 447 patients who had both cytological and histological evaluation in agreement. Cytological specimens were prepared using liquid-based cytology, and the histological result was based on subsequent surgical samples. Each specimen was digitized; on these images, nuclear morphology features were measured by the use of an image analysis system. The extracted measurements (41,324 nuclei) were separated into two sets: the training set that was used to create the RBF ANN and the test set that was used to evaluate the RBF performance. The system aimed to predict the histological status as benign or malignant.Results: The RBF ANN obtained in the training set has sensitivity 82.5%, specificity 94.6%, and overall accuracy 90.3%, while in the test set, these indices were 81.4%, 90.0%, and 86.9%, respectively. Algorithm was used to classify patients on the basis of the RBF ANN, the overall sensitivity was 95.0%, the specificity was 95.5%, and no statistically significant difference was observed.Conclusion: AI techniques and especially ANNs, only in the recent years, have been studied extensively. The proposed approach is promising to avoid misdiagnoses and assists the everyday practice of the cytopathology. The major drawback in this approach is the automation of a procedure to accurately detect and measure cell nuclei from the digitized images.

Dermatofibrosarcoma protuberans (DFSP) is a superficial mesenchymal neoplasm that originates from the dermal fibroblasts and tends to be locally aggressive. Although infrequent, it is one of the most common cutaneous sarcomas. It mainly affects young and middle-aged patients 20 to 50 years old. Any area of skin may be involved, but the most common sites of presentation are the trunk and extremities. DFSP of the breast is extremely rare. It classically presents as a nodular, exophytic, cutaneous mass, though initially it can manifest as a flat plague and can show persistent but slow growth for many years. Due to increased risk of local recurrence, the standard of care for localized disease is surgical excision with adequate margins. Wide local excision is the most common technique used, but as an alternative Mohs micrographic surgery has emerged as a procedure that offers lower local recurrence rates. Metastases are rare but have been previously reported. In such patients, treatment with imatinib or radiotherapy can be considered. The current case presents a 52-year-old lady with DFSP of the breast that was successfully managed by the Breast Unit of Athens Medical Center-Psychiko Clinic.

This article describes how colorectal carcinomas (CRC) are the fourth most frequently diagnosed cancer. However, despite advances in specific treatment, approximately 40% to 50% of patients have disease recurrence following potentially curative surgery, highlighting the demand for improvement of staging, treatment and prognosis. Inflammatory infiltration composed of lymphocytes is a common feature found in neoplasms and studies have indicated an association between tumor immune infiltrates and favorable disease outcomes. The aim of this article is to assess the association of tumor infiltrating lymphocytes and DNA damage response expression levels with disease outcome and survival in stage II and III CRC. The authors examined the prognostic value of immunological biomarkers using 5-year follow-up data to train an Artificial Neural Network (ANN) designed for the prediction of personalized risk. Data for three biomarkers which were found significant were subsequently used to train the ANN which predicted 5-year survival with 72.9% sensitivity and 68.8% specificity.

Salivary gland adenocarcinoma not otherwise specified (NOS) is a malignant epithelial tumor composed of ductal/glandular structures with or without cystic formation. Histologically it is classified as high grade with relevant biological behavior. Although both minor and major glands may be involved, the majority (60%) implicate the parotid gland. Location, regional lymph node status, and histological grade are some of the factors that predict the progress of the disease and the development of metastases. Long follow-up is considered the standard option as distant metastases (DM) may occur despite regional control. Primary sites of DM, besides lymph nodes, include bone, lung, and liver. Herein we report a unique case of a 68-year-old female with a previous history of high-grade adenocarcinoma NOS of her right parotid gland. On her biannual follow-up examination, MRI revealed an abnormal increase in the size of a known uterine leiomyoma of the posterior uterine wall. Positron emission tomography-CT (PET-CT) showed increased uptake in the uterus and lungs. On frozen section, adenocarcinoma was found at the center of the leiomyoma. Histological and immunohistochemical findings were consistent with secondary involvement by the salivary gland adenocarcinoma NOS. Treatment consisted of cyclophosphamide, adriamycin, and cisplatin with poor outcome. The patient was lost to follow-up. Review of the literature indicates that no similar case has been reported in the English literature.

The aim of this study is to compare machine learning algorithms (MLAs) in the discrimination between benign and malignant endometrial nuclei and lesions. Nuclei characteristics are obtained via image analysis and were measured from liquid-based cytology slides. Four hundred sixteen histologically confirmed patients were involved, 168 healthy, and the remaining with pathological endometrium. Fifty percent of the cases were used to three MLAs: a feedforward artificial neural network (ANN) trained by the backpropagation algorithm, a learning vector quantization (LVQ), and a competitive learning ANN. The outcome of this process was the classification of cell nuclei as benign or malignant. Based on the nuclei classification, an algorithm to classify individual patients was constructed. The sensitivity of the MLAs in training set for nuclei classification was in the range of 77%-84%. Patients' classification had sensitivity in the range of 90%-98%. These findings indicate that MLAs have good performance for the classification of endometrial nuclei and lesions.

Primary squamous cell carcinoma (SCC) of the ovary is rare. Most cases arise from a cystic teratoma or less frequently from Brenner tumor or endometriosis. We reviewed 36 cases of primary ovarian SCC reported in the literature including a case diagnosed and treated in our institution.Data was collected by using the key-words "primary squamous cell carcinoma" and "ovary" on Google Scholar and PubMed in April 2018. All reviewed cases were analyzed according to diagnosis, surgical approach, adjuvant therapy and outcome.To date 23 articles presenting 36 cases of primary ovarian SCC are reported. Nine patients had stage I, 8 stage II, 11 stage III and 5 stage IV disease, whereas 3 patients had in situ carcinoma. All patients underwent surgery (mainly hysterectomy with bilateral salpingo-oophorectomy). Adjuvant therapy was reported in 24 patients, 15 of which received chemotherapy, 6 radiotherapy and 3 a combination of both. Chemotherapy regimens were similar to the ones used in ovarian carcinoma (more often platinum plus paclitaxel). Follow-up period was in general short and survival varied between 9 days and 14 years, depending on the stage at diagnosis.Primary ovarian SCC is a rare entity with poor prognosis, compared to serous carcinoma. Treatment is usually extrapolated from classical ovarian carcinoma algorithms, including surgical management combined with adjuvant chemotherapy with or without radiotherapy. Further investigations are needed to define optimal treatment, such as chemotherapy regimens and the role of radiotherapy and lymph node dissection.

Introduction/Background Glassy cell carcinoma (GCC) of the uterine cervix is a poorly differentiated subtype of adenosquamous carcinoma. It accounts for 1–2% of cervical carcinomas. It has been associated with high-risk human papillomavirus infection (HPV 16, 18 and 32). We present a case of this rare entity. Methodology A 46-year-old patient was admitted to the gynecology department. She had a previous diagnosis of poorly differentiated carcinoma on cervical biopsy performed due to an abnormal enlargement of the uterine cervix on routine ultrasound examination. Radical hysterectomy with bilateral pelvic lymph node dissection and omentectomy was performed. On gross examination, the cervix was barrel-shaped, occupied by a bulky, white-gray exophytic mass measuring 60 mm in its greatest dimension. Results On microscopic examination, the tumor consisted of solid nests of large, pleiomorphic cells with prominent nucleoli, lacking glandular or squamous differentiation, with ground-glass or finely granular cytoplasm. Several mitoses were identified. Tumor nests were embedded in a heavy inflammatory infiltrate. Immunohistochemically tumor cells showed positive staining for EMA, Vimentin, P16, pCEA and negative for CK7, CK20, P63, ER, PR, and HMB45. The omentum and pelvic lymph nodes were tumor free. Our diagnosis based on morphological and immunohistochemical findings was GCC of the uterine cervix. The patient received adjuvant radiotherapy and chemotherapy. She remains disease-free 9 months post surgery. Conclusion GCC is a clinically aggressive subtype of cervical carcinoma with rapid growth, and early metastases. Treatment consists of surgery followed by radio-chemotherapy or neoadjuvant therapy in cases with advanced disease. Multimodal therapy has improved prognosis in patients with FIGO stage I or II but not in higher stage disease. Disclosure Nothing to disclose.

Introduction/Background Breast cancer (BC), depending on molecular subtype, usually metastasize to the liver, lungs, bone and brain. We report an interesting case of a patient, previously treated for hormone receptor-positive, HER2-negative early BC, who presented 7 years later with HER2-positive metastatic disease. Finally she developed an intramedullary spinal metastasis. Methodology A 61 year-old woman underwent left mastectomy in 2010 for BC hormone receptor-positive, HER2-negative, with lymph node involvement. She also received adjuvant chemotherapy, radiation therapy and hormonal treatment until 2017. In 2017 she received Paclitaxel/Bevacizumab followed by Bevacizumab maintenance for recurrence in the lungs, liver and bones. In February 2018 she underwent rebiopsy in the liver due to progressive disease, which revealed metastasis from hormone receptor-negative, HER2-positive BC. She received Trastuzumab/Pertuzumab/Docetaxel until August 2018. In the meanwhile she receive whole-brain radiation therapy in June 2018 for multiple brain metastases. In August 2018 she started Trastuzumab-Emtansine due to progressive disease. On January 2019 she presented in our department with paraplegia. Results Spine MRI was performed ad revealed an intramedullary lesion on level T12-L1 of 3.6×1.5×1.4 cm with contrast enhancement. Differential diagnosis included metastasis from breast cancer and ependymoma. Blood tests indicated thrombopenia and hepatic disturbances. After discussion with the neurosurgeons and radiation oncologists we did not offer any treatment to our patient, because of the established paralysis of the lower extremities. Conclusion BC can rarely give intramedullary spinal lesions. Their treatment is quite challenging. Disclosure Nothing to disclose.

Introduction/Background Adenoid cystic carcinoma (ACC) is an aggressive slow-growing tumor occurring in women with mean age 49 years. The neoplasm is similar to those of salivary gland, evolving from chromosomal translocation (NFIB) and having no HPV-correlation. ACC accounts for 15% of Bartholin´s gland (BG) malignancies and for 2–7% for vulvar carcinomas, with less than 90 cases described. Treatment remains individualistic as there is no level I evidence available, making the new cases crucial to be reported. Methodology A 46-year-old female, presented after a positive biopsy for ACC-BG. She reported a small swelling in her left posterior labia minor during the last 6 months. She had the BG cyst removed and the pathology showed ACC 3 × 3 cm encapsulated inside the cyst. Another 45-year-old female, presented after an excision of a vulvar mass 3,5 × 2,5 × 2,5 cm of the left labia minor after swelling for the last 2 years which resulted histologically in ACC-BG with positive margins and neural and muscular invasion. Results Supplementary surgery was decided and the first patient underwent radical left hemivulvectomy with ipsilateral inguinofemoral lymph node excision. There was no residual disease and no adjuvant therapy was needed. In the second case we performed imaging control and radical vulvectomy with bilateral inguinofemoral lymph node dissection. The pathology-report described a middle line tumor 2 × 1,25 × 1,2 cm infiltrating the outer third of vagina plus one left lymph node, having negative margins (FIGO stIIIa); she then underwent adjuvant radiotherapy. Both women are disease-free after 16 and 20 months respectively. Conclusion Radical excision with negative margins is beneficial, while adjuvant radiotherapy should be considered for patients with risk factors (tumor size, lymph node status, surgical margins). Lung is the most common site of distant metastasis and several chemotherapeutic agents have been used such as cyclophosphamide, adriomycin, 5-FU, cisplatin and tamoxifen. ACC should always be considered in patients older than 40 years with BG lesions. Disclosure Nothing to disclose

Introduction/Background After evaluating the Argon insufflation (99, 9%) effectiveness on improving the method of cervical tissue excision by loop electrosurgery (leep) in bovine cervical specimens we use it safely in our everyday office practice in many leep cases. (the experiment mentioned was presented in ESGO congress2015). Methodology Argon is a chemical element in group 18 of the periodic table and is a noble gas. It is colorless, odorless, nonflammable and nontoxic as a gas. Argon is chemically inert under most conditions and forms no confirmed stable compounds at room temperature. Argon constitutes a cheap material. It has low thermal conductivity and is sometimes used even for extinguishing fires. Based on the argon characteristics above and our previous experience we herein use the simultaneous argon insufflation in the area before cervix so as there is no oxygen to be burned and provoke smoke or tissue damage during leep. Results We apply a small plastic tube that isufflates argon connected to a 5-litre bottle of argon gas on the diathermy pen. The diminution of the thermal injury of the cone while using this patent is macroscopically obvious and microscopically significant. Especially, the zone of carbonization and coagulation of the entrance, middle and exit margins of the loop is decreased. Conclusion Although argon in Gynecology is mostly known in its liquid form via Cryosurgery procedures or in argon plasma beam electrosurgery the argon gas can also be used as a safe and cheap material. The use of lower watts electrosurgery allows us to excise high quality histological specimens inexpensively by altering the air medium of loop electrosurgical procedure to Argon or cooled-Argon. Disclosure Nothing to disclose

Introduction/Background Undifferentiated pleomorphic sarcoma (UPS) is a malignant mesenchymal neoplasm presenting very rarely as a primary breast tumor. We present a case of UPS of the breast with aggressive clinical behavior. Methodology A 67-year-old patient was admitted to our hospital due to a palpable lump of the right breast. Imaging studies revealed a 7 cm multilobular mass. A lumpectomy was performed. On gross examination, the tumor was solid, white-gray and had a greatest dimension of 7.3 cm. Results On microscopic examination, the tumor consisted of spindle cells with alternating cellularity, high-grade atypia, and brisk mitotic activity. Immunohistochemical stains were positive for CD68, CD163, CD10 and negative for AE1/AE3, EMA, CK7, Ck34bE12, p63, SMA, Desmin, S100, CD31, CD34, and CD56. The diagnosis of UPS of the breast was made. The surgical margins were infiltrated. A month later she underwent a modified radical mastectomy. The surgical margins were infiltrated. The patient received first-line systemic therapy. After having received one cycle of systemic therapy, the tumor recurred locally. A third surgical operation was performed to achieve local control. The patient‘s condition deteriorated very rapidly. A month later she presented with metastatic disease to the lungs. The patient passed away due to respiratory insufficiency. Conclusion Primary breast sarcoma diagnosis may occasionally prove very challenging. Before making the diagnosis other more common entities (malignant phyllodes tumor, metaplastic carcinoma and metastatic sarcoma from another primary site) have to be excluded. Extensive tumor sampling, attention to histological detail paired with the use of the appropriate immunohistochemical markers will usually help in making the diagnosis. Similar to the case we present data found in the literature indicates that breast UPS show an aggressive clinical course and a high incidence of recurrence and metastasis. Disclosure Nothing to disclose.

Introduction/Background Bowel and uterine carcinomas are often related genetically, through hereditary syndromes or as metastasizing sides. Metastasis to the female genital tract from extragenital malignancies are rare, and the prognosis is extremely poor. Methodology We herein report a case of a 62 years old woman with metastatic carcinoma to the uterine body from a colon adenocarcinoma, presenting with vaginal bleeding. 16 months before she had an urgent laparotomy due to obstructive ileus. She underwent right colectomy, right oophorectomy and lymphadenectomy and the histological results reported a colon-cecum adenocarcinoma (pT4aN0 grade2) up to the pericolic fat. The appendix, the right adnexa and the lymph nodes were negative. The imaging showed no metastasis to another organ. She received 9 cycles of chemotherapy with oxaliplatin and capecitabine (Xelox) and was followed up. The diagnostic curettage that followed revealed a moderate adenocarcinoma of the uterus compatible with the colon adenocarcinoma (CK7 (+), CK20 (+), p16 (+), CDX2(+), ER (−), PR (−)). Results Colonoscopy-biopsies were negative for malignancy and PET-CT reported a large uterus with focal disease and suspicious pelvic lymph nodes. The rest body scan was negative. She underwent wide total hysterectomy and left oophorectomy, excision of the mesorectum and parts of peritoneum, omentectomy and pelvic lymphadenectomy. The pathology examination reported the uterine adenocarcinoma infiltrating the serosa and myometrium. The mesorectum and 3/11 left lymph nodes were positive. The endometrium was atrophic and the left adnexa, the omentum and peritoneal parts excluded were negative for disease. The patient received 8 cycles of XELIRI-protocol (Capecitabine plus 3-weekly irinotecan) and is now -14 months after- free of relapse. Conclusion According to our findings the tumor was developed of metastases in tissue continuity. To choose the best treatment, it is necessary to always diagnose whether a uterine bleeding is due to a primary uterine or a metastatic carcinoma. Disclosure Nothing to disclose.

Introduction/Background Invasive lobular carcinoma (ILC) with extracellular mucin production is a recently described new variant of ILC with few reported cases in the English literature. We report another case of this rare entity diagnosed in the pathology department of Saint Savvas hospital. Methodology A 65-year-old patient was admitted to the hospital for a palpable lump of the right breast. Physical examination and imaging studies were consistent with malignancy. Frozen section was positive while sentinel lymph node biopsy was negative for malignancy. A right mastectomy was performed. On gross examination the tumor was soft, pale, grayish blue, gelatin-like and well circumscribed. On microscopic examination the tumor consisted of two foci measuring 13 and 6 mm. It consisted of a non-mucinous ILC component of the classical and solid type. The mucinous area represented about 70% of the tumor. Extracellular mucin production was seen in the form of relatively circumscribed multiple nodular areas. Tumor cells in the mucinous area were arranged in clusters and single cells. Several signet ring cells were identified. Tumor cells showed positive staining for ER and negative for PR, HER-2 and E-Cadherin. Ki-67 stained 25% of tumor nuclei. Results The diagnosis of ILC with extracellular mucin production was made. Adjuvant radiotherapy and hormonal therapy was administered. The patient is alive without evidence of recurrence or metastasis six months after surgery. Conclusion Awareness of this new entity will help us avoid confusion with other mucin-producing neoplasms. Differential diagnosis includes mucinous carcinoma, solid papillary carcinoma with extracellular mucin production and composite mucinous and lobular carcinoma. Correct diagnosis is important because both treatment and prognosis are distinct. Disclosure Nothing to disclose.

Introduction/Background Primary squamous cell carcinoma of the ovary (PSCCO) is a rare entity. Most squamous cell carcinoma cases arise from a cystic teratoma or less frequently are associated with Brenner tumor or endometriosis. We review 36 cases of PSCCO reported in the literature. Methodology Data were collected in November 2018. Twenty-three articles from 1964 to 2018 presenting thirty-six cases of PSCCO are reported in the literature. All reviewed cases were analyzed according to diagnosis, surgical approach, adjuvant therapy and outcome. Results Patients‘ age ranged from 27 to 90 years (mean age: 52.9 years). Tumor size ranged from 1.5 to 26 cm in the greatest axis (mean value: 10.3 cm). The most frequent symptom was abdominal pain. Other symptoms were related to tumor growth, metastasis, local extension or were non specific. Only few patients were asymptomatic. In 10 cases high grade Cervical Intraepithelial Neoplasia was associated. Grossly tumors were either cystic or solid. Microscopically several different morphological patterns were present. All patients were treated surgically, in most cases with hysterectomy and bilateral salpingo-oophorectomy. Adjuvant therapy was administered in twenty-four patients. Information regarding outcome was available in 31 cases. Follow-up time ranged from 9 days to 90 months. Eleven patients were alive with no evidence of disease, three were alive with metastases and seventeen succumbed to disease. Conclusion PSCCO is a rare entity. Treatment includes combination of surgical management with adjuvant therapy when necessary. Its prognosis seems to be poorer compared to other ovarian carcinomas. Further investigations are needed to define optimal treatment and prognosis. Disclosure Nothing to disclose.

Invasive lobular carcinoma of the breast is the second most common subtype of breast carcinoma. It accounts for 5-15% of the breast carcinoma cases reported. It shows a different metastatic pattern compared to invasive carcinoma of no special type. There are several variants of this cancer including the classic, solid, signet ring cell, tubulolobular, alveolar, trabecular, pleomorphic, and mixed subtypes each one with a distinctive morphology. Invasive lobular carcinoma has been associated with intracellular mucin production, in the form of intracytoplasmic lumina and signet ring cells whereas extracellular mucin production has been related to carcinomas of ductal origin. A new variant that displays extracellular mucin production was described recently. Only nineteen cases of this rare entity have been reported to date. In this manuscript, we report an additional case of invasive lobular carcinoma with extracellular mucin production, describing its clinico-pathological characteristics, and review the literature on the same.

Matrix-producing carcinoma (MPC) is a rare subtype of metaplastic breast carcinoma (MBC) that was first described in 1989 by Wargotz and Norris. It accounts for less than 1% of breast carcinomas and has distinctive clinical, morphological, and immunohistochemical features. Histologically it consists of invasive carcinoma of no special type with transition to cartilaginous or osseous matrix without a spindle cell component. Data on this entity are limited with the literature consisting mostly of case reports and a small number of case series. We report a case of matrix-producing breast carcinoma, with excellent clinical outcome. We also discuss the histogenesis, imaging, histological, and immunohistochemical characteristics, treatment, and focus on the differential diagnosis of this rare tumor.

Background Pericardial synovial sarcomas (PSS) are very rare tumors, with dismal prognosis and limited data. We describe the clinical features and identify prognostic factors of primary PSS. Case report We describe the case of a 56-year-old male patient with PSS managed by the multidisciplinary team of thoracic oncology. The therapeutic plan comprised surgery, chemotherapy, stereotactic radiosurgery and targeted therapy, with excellent results. Materials and methods Data from 37 cases reported in English during the past 20 years were gathered and analyzed. PSS was found to occur at a mean age of 36±17.082 (range=13-67) years. Survival analysis was performed on 20 cases with follow-up of at least 6 months. Conclusion Only complete resection of the tumor seems to be an independent prognostic factor. To our knowledge, this is the first report on the safety and effectivity of pazopanib in PSS and may provide guidance for similar cases in the future.

Ewing Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) is a malignant small round cell tumor belonging to the Ewing Sarcoma Family of Tumors. It occurs more commonly in children and young adults. Its localization in the adrenal gland is extremely rare. We reviewed 35 cases of ES/PNET of the adrenal gland reported in the literature and presented our case.Data were collected by searching for ES/PNET and adrenal gland key words on Google Scholar and PubMed in March 2018, including a case diagnosed in our department. We analyzed all reviewed cases for diagnosis, surgical and systemic therapy and outcome.To date 24 articles presenting cases of ES/PNET of the adrenal gland are reported in the literature. We included in our review 35 cases previously described and one new case. Histologically all cases consisted of sheets of small round cells. Immunohistochemistry was also performed in all cases. Most cases stained positive for CD99 and negative for lymphocytic markers. Markers of epithelial differentiation displayed variable results. In all cases tested, characteristic translocations were displayed supporting the diagnosis. All patients but four were treated surgically and the majority received adjuvant therapy. Only very few cases received neoadjuvant chemotherapy.Primary ES/PNET of the adrenal gland is a rare tumor, showing specific morphological, immunohistochemical and cytogenetic characteristics. Treatment consists of surgery, chemotherapy and radiotherapy. Further investigations paired with long term follow-up are necessary to define prognosis for this rare entity.

Immunotherapy (IO) with anti-PD1 inhibitors is available for the treatment of recurrent/metastatic squamous cell carcinomas of the head and neck (SCCHD) since 2016. Both nivolumab and pembrolizumab were tested in phase 3 randomized trials in adults progressing on or after platinum-based therapy and were found to confer an overall survival benefit compared to investigator’s choice. However, very limited data exist concerning IO use in rare subtypes of head and neck carcinoma, like salivary gland carcinoma. We retrospectively collected clinical data of all patients diagnosed with rare subtypes of head and neck carcinoma, who were treated with immune checkpoint inhibitors in our department during the last 5 years. We analyzed safety and efficacy of these therapies. We identified six patients who received nivolumab for recurrent or metastatic head and neck carcinomas, between 31 and 57 years old. All patients had received at least one line of platinum-chemotherapy, as well as radiation therapy. Treatment was administered every 2 weeks, at a dose of 3 mg per kilogram of body weight. Number of nivolumab cycles varied between 2 and 18. Progression-free survival varied from 1 to 12 months and overall survival from 4 to 24 months. Tolerance was very good, except for one case of diabetes and hypothyroidism requiring medication. There is currently insufficient evidence regarding the optimal treatment of the rare non-squamous cell carcinoma of the head and neck. Our case series supports a role for immunotherapy in these patients. However, larger collaborative studies are needed to evaluate this treatment.

Immunoglobulin G4-related disease is an immune-mediated fibroinflammatory disease with single or multiple organ involvement. Clinically it mimics several benign and malignant tumors, as well as infectious, and inflammatory disorders. It typically presents as multiple tumor-forming lesions. Histological and immunohistochemical findings are characteristic. Serum immunoglobulin G4 levels are usually increased. Systemic corticosteroid administration is the treatment of choice with good response, especially in early disease stages. We present the first case of immunoglobulin G4-related disease presenting as an isolated tumor forming lesion of the left pterygopalatine fossa. Imaging studies indicated a benign process. Histological findings were consistent with IgG4-related disease. The patient showed a good response to systemic corticosteroid treatment and remains free of symptoms 18 months following diagnosis.