Pleomorphic Type Research Articles

The Great Mimicker: Goblet Cell Adenocarcinoma of the Appendix Mimicking Mammary Carcinoma in a Patient with a History of Metastatic Breast Cancer

Abstract Introduction/Objective Goblet cell adenocarcinoma of the appendix is a rare and aggressive malignancy with high- grade histological patterns that pose diagnostic challenges due to their resemblance to other tumors. Previous reports have indicated that high-grade goblet cell adenocarcinoma may exhibit similarities to well-differentiated neuroendocrine tumors, and metastatic high-grade goblet cell adenocarcinoma can manifest diverse histologic growth patterns within a single section. Methods/Case Report We present a case of an 82-year-old female with a known history of breast cancer with sacral metastasis, presenting with a left upper quadrant “pinching” sensation. Histopathological examination following colectomy showed features of metastatic mammary carcinoma with mixed lobular (pleomorphic and partial signet ring cell type) and ductal features (Figure 1); Notably, different histologic patterns were observed in two tumor deposits, highlighting the complexity of the diagnosis (Figure 2). However, further investigation of the tip of the appendix revealed goblet-like mucinous cells arranged in a tubular growth pattern (Figure 3). Immunohistochemical stains were performed with adequate controls, revealing positive staining for CK20, SATB2, and CDX2, while showing negativity for BRST2, SATB2, GATA3, CK7, PR, ER, and HER2 (Figure 4). These findings corroborate the diagnosis of goblet cell adenocarcinoma. Results (if a Case Study enter NA) NA Conclusion In our case, we observed morphological features of goblet cell adenocarcinoma that overlapped with those of established mammary carcinomas. This scenario underscores the diagnostic challenges posed by rare tumors and emphasizes the significance of identifying the low-grade component of goblet cell adenocarcinoma, particularly in patients with a history of metastatic breast cancer.

Comparison of Clinicopathological Features of Pleomorphic and Invasive Lobular Breast Carcinomas.

Accounting for about 15% of invasive lobular carcinomas and 1% of breast carcinomas, pleomorphic lobular carcinoma is known to be a rare histological subtype of invasive lobular carcinoma. Yet, it is more aggressive and produces a worse prognosis than other breast cancers. Ultimately, the present study compares the clinicopathological features of pleomorphic and invasive lobular breast carcinomas. In the study, we retrospectively evaluated the data of 262 patients with histological subtypes of classical and pleomorphic lobular cancers having been recruited for surgical operations. After resorting to Kolmogorov-Smirnov and Shapiro-Wilk tests to check the normality of distribution, the categorical and continuous variables were compared between the groups using the chi-square test and independent samples t test, respectively. In all analyses, we considered a P-value of <.05 to be statistically significant. Our findings revealed that the groups with lobular and pleomorphic groups significantly differed by Ki-67 value, estrogen receptor negativity, grade, multicentricity, multifocality, surgical margin positivity, completion mastectomy, and metachronous contralateral tumor (P < .05). We discovered that pleomorphic type was associated with higher grades, estrogen receptor negativity, and Ki-67 expression. The incidence of metachronous breast cancer was high in the pleomorphic group, which may be a noteworthy finding to be considered in follow-ups. In addition, the high rates of multicentricity and multifocality of tumors in the pleomorphic group may be associated with increased surgical margin positivity and a higher likelihood of mastectomy. In a nutshell, our findings may guide patients and surgeons regarding the type of intervention and reconstruction options to be adopted in prospective surgeries.

Differences in Histological Subtypes of Invasive Lobular Breast Carcinoma According to Immunohistochemical Molecular Classification.

The aim of this study was to compare the differences between histological and molecular subtypes of invasive lobular carcinoma (ILC) of the breast, in order to be able to predict the behavior and prognosis of the disease, as well as to effectively determine therapy. This study included 263 cases of breast ILC diagnosed over a seven-year period. The diagnosis of invasive lobular carcinoma is based on the characteristic growth pattern and phenotype of cancer cells with the respective subtypes: classic, alveolar, solid, tubulolobular, pleomorphic and mixed lobular type. The examined cases were divided into five groups according to molecular classification based on the expression of ER, PR, HER2 and Ki67 immunohistochemical markers. It was found that the pleomorphic subtype occurred statistically significantly less often as the luminal A subtype compared to others (p = 0.00027), and the HER2-enriched subtype occurred statistically significantly more often in the pT4 stage (p = 0.024). The results of this study significantly singled out the luminal A subtype, and among them classic ILC, as the subtype with the most favorable expression ratio of the investigated predictive/prognostic immunohistochemical markers.

Unraveling the Clinicopathological Diversity and Subtypes of Rhabdomyosarcoma: A Study From a Tertiary Care Center.

Background and objective Rhabdomyosarcoma (RMS) is a rare and malignant mesenchymal tumor characterized by skeletal muscle differentiation. While it is a common soft tissue sarcoma in children, its incidence significantly decreases with advancing age, rendering it exceptionally rare in individuals aged more than 45 years. This study aimed to shed light on the clinicopathological diversity and subtypes of RMS, thereby providing a comprehensive overview for enabling diagnostic precision and therapeutic strategies in treating this infrequently encountered malignancy in adults. Methodology This was a hospital-based cross-sectional studyconducted in the Department of Pathology. Patients who were diagnosed with RMS over a period of three years were included in the study. The demographic features such as age and sex and aspects related to the tumor site, size, subtypes of RMS, and immunohistochemical expression were studied. Results A total of 14 cases were included in our study. The age at diagnosis ranged from four months to 65 years with a male-to-female ratio of 1:2.5. The sites of presentation were head and neck, trunk, pelvis, genitourinary tract, and retroperitoneum. The histological types were embryonal, alveolar, pleomorphic, and mixed and spindle cell types. The tumor cells were positive for immunohistochemistry markers desmin, MyoD1, and vimentin. Conclusion This study delved into the clinicopathological intricacies of RMS, offeringcomprehensive insights into its diverse subtypes. Our findings underscore the unique presentation of RMS in adults, with trunk and genitourinary tracts emerging as primary sites and alveolar and pleomorphic RMS observed as the predominant histological subtypes. Furthermore, the study sheds light on rare subtypes with distinct anatomical distributions.

Expression of PD-L1 in breast invasive lobular carcinoma.

The purpose of this study was to investigate the expression of PD-L1 in invasive lobular carcinoma (ILC) and to determine its implications. Tissue microarrays were constructed for 101 cases of ILC, and immunohistochemical staining for PD-L1 (using 22C3, SP142, and SP263 antibodies) was performed to examine the correlation between staining results and clinicopathologic parameters. The positive cut-off values were defined as tumor cell (TC)≥1%, immune cell (IC)>0%, and IC≥1%. The range of PD-L1 TC positivity was 0.0-2.0%, with PD-L1 SP263 TC showing the highest positivity of 2.0%. The range of PD-L1 IC positivity was 0-21.8% for IC ≥ 1%, with PD-L1 22C3 IC showing the highest positivity. When PD-L1 IC was positive (IC≥1%), the highest antibody agreement was observed between SP263 and SP142 (OA = 93.1%), while the lowest agreement was observed between 22C3 and SP263 (OA = 73.3%, κ = 0.040). PD-L1 22C3 IC positivity (≥1%) was associated with high nuclear grade (p = 0.002), HER-2 positivity (p = 0.019), and pleomorphic type (p = 0.002). PD-L1 expression in ILC shows a low TC positivity rate (0-2%) with various antibody clones and a variable IC positivity rate (0-21.8%). Pleomorphic type ILC exhibits higher PD-L1 IC positivity.

Surgical management of giant recurrent retroperitoneal liposarcoma: a case report and review of the literature.

A 59-year-old female patient consulting for a large abdominal mass. The abdominal computed tomography showed three retroperitoneal masses, and the surgical exploration revealed a huge process in the retroperitoneal cavity, which takes the left renal compartment, and the left colon. The intervention consisted of a mono-bloc excision of the mass taking the spleen, the left renal compartment, and the left colon with colonic anastomosis. The histological examination concluded the existence of a well-differentiated myxoid liposarcoma of grade I, the postoperative follow-up was simple. One year later, she underwent an excision of a recurrence of the same retroperitoneal location, but of a pleomorphic cell histological type of grade II according to the FNCLCC classification. We review the literature, the pathological, therapeutic, and prognostic aspects of this tumor. Retroperitoneal liposarcoma is a rare tumor. Its gravity is due to an often-late diagnosis, a complete imaging workup including ultrasound, computed tomography and often MRI is necessary preoperatively to determine the relationship with the different organs. The definitive diagnosis is histological, surgery is the most effective treatment and can be extended to neighboring organs. the frequency of recurrence requires particular surveillance. We highlight the importance of radical surgical excision to avoid retroperitoneal liposarcoma tumor complications and to minimize the recurrence risk.

Liposarcoma of the pleural cavity

Liposarcoma rarely occurs in the pleura or thoracic cavity, and few reports appear in the literature. We hypothesized that combining clinicopathologic, immunohistochemical, and fluorescence in situ hybridization methods would allow definite diagnoses. Using formalin-fixed, paraffin-embedded blocks, we examined 6 atypical lipomatous tumor/well-differentiated liposarcomas (ALT/WDLPS), 5 dedifferentiated liposarcomas (DDLPSs), 2 pleomorphic liposarcomas, and 1 myxoid liposarcoma (MLPS). We used the Kaplan-Meier method and the Wilcoxon test for survival analysis for prognostic factor evaluation. Histologically, ALT/WDLPS was composed of a relatively mature adipocytic proliferation, accompanied by some lipoblasts. DDLPS exhibited round-to-oval tumor cells with a high nucleus-to-cytoplasm ratio that had proliferated in nests, accompanied in case 10 by some giant cells but no fatty cells. The pleomorphic type contained a varying proportion of pleomorphic lipoblasts. MLPS displayed uniform round- to oval-shaped cells and small signet-ring lipoblasts in a myxoid stroma. Immunohistochemically, 11 (79%), 11 (79%), and 10 (71%) of 14 cases were positive for S-100, p16, and CDK4, respectively. Sixof the 14 cases (43%) were positive for MDM2and adipophilin. One case of ALT/WDLPS and 3 cases of DDLPS exhibited MDM2 amplification by fluorescence in situ hybridization (Vysis LSI MDM2 SpectrumGreen Probe plus Vysis CEP 12 SpectrumOrange probe). ALT/WDLPS was the most favorable type for survival, while adipophilin tended to be a negative prognostic factor for pleural liposarcoma. For a firm diagnosis of liposarcoma in the pleura, immunohistochemistry for CDK4, MDM2, and adipophilin together with MDM2 gene amplification by fluorescence in situ hybridization may be an important diagnostic tool.

Pleomorphic type undifferentiated gastric sarcoma, report of a case.

Reports on pleomorphic type of undifferentiated sarcoma (PUS) originating from the gastrointestinal tract, especially the stomach, are extremely rare. We herein report a case of pleomorphic type undifferentiated gastric sarcoma. The patient was a 67-year-old woman. The chief complaint was upper abdominal pain. Upper gastrointestinal endoscopy, ultrasonography, and contrast-enhanced computed tomography showed two submucosal tumors at the greater curvature of the fundus and the lesser curvature of the gastric angle. Endoscopic ultrasound-guided fine-needle aspiration revealed a c-kit-negative spindle cell tumor at the greater curvature of the fundus. Total gastrectomy, splenectomy, and partial resection of the diaphragm and liver were performed. One lesion had invaded the lateral segment of the liver, left diaphragm and spleen. The postoperative course was uneventful. Histopathological and immunohistochemical examinations of the resected specimen revealed PUS. Peritoneal dissemination was detected at 8months after surgery. However, no effective therapeutic agents were adopted for chemotherapy. The patient had poor performance status due to disease progression and underwent best supportive care. The patient died 10months after surgery. This case highlights the imaging, histological diagnosis, and treatment strategy for PUS originating from the stomach. Surgeons should be aware of PUS as a differential diagnosis in cases with submucosal tumor of the stomach.

Retroperitoneal Liposarcoma: The Giant Type

Retroperitoneal tumors (RPTs) are very rare tumors that arise in the large space of the retroperitoneum. About two-third of these tumors are malignant, of which soft tissue sarcoma (STS) is the most common and comprises almost one-third of malignant RPTs. Twenty to thirty percent of RPTs are benign. The retroperitoneal cavity has a very large potential space for tumor enlargement to a very high diameters without causing specific symptoms, especially during the initial phase of tumor enlargement. On diagnosis, the average tumor weight is 15 - 20 kg and tumor diameter is 20 - 25 cm. The most common retroperitoneal sarcoma type is liposarcoma, which account for 20% of all sarcoma types and 40% of all retroperitoneal sarcomas (RPSs). Other less common STS arise in the retroperitoneum include leiomyosarcoma and undifferentiated pleomorphic type. Giant liposarcoma is usually defined either as tumor diameter of 30 cm or more or tumor weight of 20 kg or higher. This specific type of sarcoma is very uncommon, with few case reports published in the English literature. Herein, we present a case of a healthy 33-year-old male patient, who was admitted due to abdominal distension and increased body weight since few months. An abdominopelvic computed tomography (CT) scan demonstrated a giant retroperitoneal mass of almost 40 cm in diameter in its largest dimension, located in the right retroperitoneal space. Ultrasound (US)-guided fine needle biopsy (FNB) was consistent with well differentiated liposarcoma. Surgical resection of the tumor along with the right colon, right ureter and kidney, third and fourth duodenal parts and part of the right iliopsoas muscle was contemplated. Histopathological report revealed well-differentiated liposarcoma of 50 cm in diameter, with foci of dedifferentiation, presented by pleomorphic sarcoma. Surgical margins were microscopically negative.

A Case of Plasma Cell Myeloma with Multilobated and Monocytoid Morphology

Plasma cell myeloma (PCM) accounts for approximately 1% of malignant tumors and 10 to 15% of hematological neoplasms. It is a malignant disease characterized by abnormal proliferation of plasma cells and monoclonal immunoglobulins or free light chains (FLCs). There are many morphological variations of the myeloma plasma cells which include mature, immature, plasmablastic, and pleomorphic types. Here, we report a rare presentation of PCM with normal serum protein electrophoresis but elevated serum FLCs and having convoluted, multilobated, and monocytoid morphology. This type of morphology is related to an aggressive clinical course and resistance to conventional chemotherapy. Moreover, absence of M protein in serum/urine electrophoresis does not rule out the diagnosis of PCM and serum FLC assays plays an important role in this kind of scenarios.

Clinicopathological and molecular genetic features of cyclin D1-negative mantle cell lymphoma

Objective: To investigate the clinicopathological features and molecular genetics of cyclin D1-negative mantle cell lymphoma (MCL). Methods: The clinicopathological features and molecular genetics of CyclinD1-negative MCL diagnosed between January 2016 and July 2021 at the First Affiliated Hospital of Zhengzhou University were analyzed using immunohistochemistry and fluorescence in situ hybridization. Clinical information was collected and analyzed. Results: A total of five Cyclin D1-negative MCL cases from all 212 MCL patients (5/212, 2.4%)were included. There were three male and two female patients,age ranged from 59 to 70 years (median 64 years). All patients presented with nodal lesions. None of the patients had B symptoms but four had bone marrow involvement. Histopathologically, four cases were classic MCL and one case was pleomorphic variant type. All five cases were negative for Cyclin D1 but SOX-11 were positive in all cases. CD5 was positive in four cases and one case was weakly positive for CD23. CD10 and bcl-6 were negative in all cases. CCND1 translocation was identified in three cases and CCND2 translocation in one case by FISH analysis. However,CCND3 translocations were not found in the five cases. Conclusions: Cyclin D1-negative MCL are uncommon, its accurate diagnosis needs combined analysis with morphologic and immunophenotypic characteristics and genetic changes. It may be particularly difficult to distinguish from other small cell type B cell lymphomas. FISH analyses for CCND1/CCND2/CCND3 translocations and immunohistochemistry for SOX-11 are helpful to resolve such a difficult distinction.

AN INTEGRATED APPROACH TO THE MORPHOLOGICAL DIAGNOSIS OF DIFFERENT TYPES OF PLEOMORPHIC ADENOMAS OF THE SALIVARY GLAND: LONG-TERM RESEARCH RESULTS.

The aim: To describe an integrated approach to the morphological diagnosis of different types of pleomorphic adenomas of the salivary gland. Materials and methods: Surgical and biopsy material from 30 patients with pleomorphic adenomas of epithelial, mixed and mesenchymal variants was studied using histological, immunohistochemical, genetic, morphometric and statistical methods. Results: The results of research allowed us to identify methods for determination the pleomorphic adenomas types. The first method requires an immunohistochemical reaction with a monoclonal antibody to human papillomavirus type 16, followed by counting the percentage of positively stained cells in the tumor. Thus, the mesenchymal variant of the tumor is diagnosed when the percentage of positively stained cells is < 40%. In the mixed variant, this indicator is ≥ 40%, but ≤ 70%, and in epithelial variant - > 70%. The second method was based on the multivariate discriminant analysis. Three formulae were derived to determine the tumor types (Fmesenchymal = - 41.03 + 4.96Х1 + 1.11Х2, Fepithelial = - 22.27 + 3.46Х1 + 0.85Х2, Fmixed = - 122.25 + 5.63Х1 + 3.2Х2, here Х1 - number of vessels, Х2 - specific volume of parenchyma). Conclusions: The authors identified several methods for determining the histological variants of pleomorphic adenomas. These methods will improve the morphological diagnosis of pleomorphic adenomas variants in the preoperative and postoperative periods.

Genomic assays for lobular breast carcinoma

One of the current challenges in breast cancer is the appropriate treatment of invasive lobular breast cancer (ILC) and defining the high-risk group within ILC. The biological character of ILC typically translates to a good prognosis, however, several studies have indicated that the long-term prognosis is worse than for patients diagnosed with the more commonly invasive ductal carcinoma. Many genomic tests are now available to determine whether those patients are at high risk (HR) and enable tailored treatment. Unfortunately, most of the studies in which these genomic tests have been evaluated entail retrospective analysis of a prospective trial. This review focuses on the validation of the available genomic assays based on trials performed in ILC patients, where in some instances, the various subtypes of ILC (classical, pleomorphic, and non-classic type) were taken into account. Using Oncotype DX in retrospective studies, only 1.3%-8% of ILC tumors were categorized as HR tumors. For MammaPrint, 24% of patients were classified as HR, which was associated with poor outcome. In a recent sub-analysis of the MINDACT study comprising 487 ILC patients, 16.2% were high genomic risk. EndoPredict, Prosigna Breast Cancer Prognostic Gene Signature Assay, and the Breast Cancer Index have been validated in patients receiving only endocrine treatment. Although ILC accounts for the second most common breast cancer subtype in women, none of these tests encompass tumor morphology in their algorithms. Prospective studies on ILC with genomic assays are warranted given the various subtypes of and treatment options for this underestimated, but frequently occurring cancer. Genomic assays can be employed in ILC patients to predict the risk of recurrence and identify those patients who might benefit from chemotherapy in addition to their standard treatment regimen.

Paratesticular Embryonal Rhabdomyosarcoma (RMSE-PT): A Case Study in the Surgery Department of the Regional Hospital of Ziguinchor (Senegal)

<i>Introduction:</i> Paratesticular embryonal rhabdomyosarcoma (RMSE-PT) is a rare and aggressive malignant mesenchymal tumor developed at the expense of connective tissue, representing approximately 7% of all rhabdomyosarcomas. Embryonic and alveolar histological types are most common in children, the pleomorphic type occurs exclusively in adults, and the prognosis is poor. The treatment method consists of tumor resection surgery, radiotherapy, and/or adjuvant chemotherapy. Here, we report a case of paratesticular embryonal rhabdomyosarcoma in a 3-year-old child. Through this observation, we discuss the diagnostic and therapeutic. <i>Methods:</i> In this report, our patient was a 3-year-old child with a large, painless right purse that had evolved over four months and was gradually increasing in volume. Physical examination detected a right scrotal mass, hard, painless, necrotic, suggestive of gangrene, while the left testicle was palpated at the bottom of the bursa, of normal appearance. The diagnosis revealed scrotal gangrene. <i>Results:</i> During the surgery, a large inguinoscrotal tumor was realized, and the realization of a right orchidectomy by the scrotal way with complete excision and dissection of an inguinal lymph node was finally made. The tumor markers (beta-human chorionic gonadotropin and alpha-fetoprotein) were negative, except for lactate dehydrogenase (LDH), which was increased. The surgical specimen's immunohistochemistry revealed an embryonic rhabdomyosarcoma invading the spermatic cord. The ultrasound revealed a tumor mass in the right testis. Computed tomography performed one month after surgery was normal. <i>Conclusions:</i> The care is multidisciplinary, combining surgery, polychemotherapy, and radiotherapy. The rarity of the clinical and the delay in diagnosis were the aggravating factors in the prognosis of our patient.

S1559 A Huge Surprise: Osteoclastic Giant Cell Tumor of the Pancreas

Introduction: Cystic lesions of the pancreas are often found incidentally on routine imaging. We describe an incidentally discovered pancreatic abnormality which was later confirmed to be a rare malignancy. Case Description/Methods: A 71-year old male with Warthin’s tumor and non-insulin dependent diabetes underwent computerized tomography (CT) of the soft tissues of the neck and the sinuses, incidentally demonstrating ground glass lung opacities. CT of the thorax then revealed sub-centimeter pulmonary nodules and an incidental 2.5 cm cystic mass at the pancreatic head, with biliary and pancreatic duct dilation. A pancreatic protocol CT confirmed a necrotic mass in the pancreatic head measuring approximately 3 cm, with pancreatic duct dilation up to 5 mm in the pancreatic neck. Scout EGD was unremarkable. Echoendoscopy was performed demonstrating a multi-cystic head of pancreas lesion measuring 2.8 cm x 2.8 cm, which was sampled with fine needle biopsy. Histology of the sample confirmed osteoclastic giant cell tumor, with immunohistochemistry showing cells positive for CD68, CD4, CD45, and vimentin, and negative for cytokeratin and pan-keratin. The patient underwent uncomplicated pancreaticoduodenectomy (Whipple’s procedure) with negative margins. The gross specimen (3.2 x 3.1 x 2.9 cm) confirmed undifferentiated carcinoma with osteoclast like giant cells. Discussion: Pancreatic adenocarcinoma is the most common type of pancreatic neoplasm, typically involving the pancreatic head. Pancreatic giant cell tumors (PGCT), officially termed “undifferentiated carcinoma with osteoclast-like giant cells) by the WHO, occur with an incidence of less than 1% of all pancreatic tumors. PGCTs rarely involve the pancreatic head, and typically occur in the body or tail of the pancreas. Typically occurring between the 6th and 7th decade of live, there is no preponderance between sexes. Three types are known: osteoclastic, pleomorphic, and mixed. Osteoclastic PGCT is thought to have the most favorable prognosis. Immunohistochemistry is critical in differentiating between osteoclastic and pleomorphic types, as pleomorphic GCT – typically positive for vimentin – are aggressive and more likely to metastasize. This patient did well with en-bloc surgical resection alone, though radiation therapy is sometimes offered depending on tumor size. In summary, we describe a rare pancreatic malignancy that was incidentally detected, diagnosed by EUS-FNB and successfully managed surgically.Figure 1.: a) CT pancreas demonstrating head of pancreas (HOP) lesion b) HOP mass on echoendoscopy c) gross specimen.

Pazopanib in rare histologies of metastatic soft tissue sarcoma.

BackgroundUncommon histopathological subtypes account for less than 5% cases of soft tissue sarcoma (STS) and unclassified STSs comprise another 16%, these are often chemotherapy-resistant, with a dismal outcome in unresectable/metastatic disease. Prospective studies on the use of pazopanib in this cohort of patients are lacking in the literature. Here, we describe the safety and efficacy of pazopanib in rare histologies of advanced STS.Materials and methodsWe conducted a retrospective study at two tertiary cancer centres in India, evaluating 33 cases of rare subtypes of STS, who received pazopanib as per institutional protocol between January 2013 and December 2019. Patients who received pazopanib for unresectable/metastatic disease were enrolled in this study for clinicopathologic features, treatment outcome and evaluation of prognostic factors.ResultsOut of 33 patients, there were seven cases of undifferentiated pleomorphic sarcoma, four cases each of myxofibrosarcoma, epithelioid sarcoma and malignant peripheral nerve sheath tumour, three cases each of haemangiopericytoma and spindle cell sarcoma, two cases of haemangioendothelioma and a case each of clear cell sarcoma, retroperitoneal sarcoma, angiosarcoma and pleomorphic rhabdomyosarcoma-adult type. The objective response rate was 27%. Most of the patients (67%) received pazopanib in second or subsequent lines of therapy. The majority (70%) were started at a lower dose of 400/600 mg and only 43% of these (10/23) could be escalated to a full dose of 800 mg based on tolerance. On univariate analysis, pazopanib’s starting dose didn’t predict progression-free survival (PFS)/overall survival (OS)/response rate. At a median duration of follow-up of 18.8 months (range 1.9–150.4 months), the median PFS and median OS were 10.3 months (95% confidence interval (CI): 5.9–14.8) and 17.8 months (95% CI: 10.7–29.3), respectively. 27% of the patients experienced grade ¾ toxicities, 12% required dose modification of pazopanib and 21% needed permanent discontinuation due to toxicity.ConclusionOur study shows that pazopanib is active in rare subtypes of STS.

Immunohistochemical expression of cyclin-dependent kinase inhibitors p16 and p57 in rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is a highly malignant cancer and is the most common soft tissue sarcoma in children and adolescents, but it is rare in adults (<1% of all adult malignancies). Altered expression and molecular abnormalities of cell-cycle-regulatory proteins are one of the most prominent features in RMS. Therefore, we evaluated the expression of cyclin-dependent kinase inhibitors p57 and p16, as well as p16 methylation status, along with clinicopathological characteristics and overall survival (OS) in RMS patients. This analysis was conducted on 23 pediatric and 44 adult patients. There was a male predominance in both groups and extremities were the most frequent tumor site. In adults, alveolar and pleomorphic types were almost equally represented. The majority of pediatric tumors were low grade, whereas, in adults, only one patient had a low-grade tumor. Seven pediatric (30.43%) and eight adult (18.18%) patients had a low p16 expression. The analysis of methylation status of the p16 promoter showed the presence of methylated allele only in one sample with pleomorphic histology. Six (26.1%) pediatric and 15 (34.1%) adult patients had low p57 expression, while in 17 (73.9%) pediatric and 29 (65.9%) adult patients it was assessed as high. Ninetyone percent of the pediatric patients and 32.6% of adults were alive at the end of the observational period. In adults, significant associations were found between OS and age (P = 0.020), gender (P = 0.027), tumor size (P < 0.001), lymph node status (P < 0.001), presence of metastases (P = 0.015), and p57 expression (P = 0.039). Stratification by histological type showed the correlation of low p57 expression (P = 0.030) and worse OS of patients with alveolar RMS. Univariate analysis identified age > 50 yrs. (HR 2.447), tumors > 5 cm (HR 21.31), involvement of regional lymph nodes (HR 3.96), the presence of metastases (HR 2.53), and low p57 expression (HR 2.11) as predictors of lower OS. Tumor size, regional lymph nodes involvement, and metastases were the independent predictors after multivariate analysis, while p57 did not predict OS in an independent way. In summary, although p57 was not confirmed to be an independent predictor of OS, our results indicate that its low expression may be the marker of aggressive phenotype and poor prognosis in adult RMS patients. Also, our findings suggest that epigenetic inactivation of p16 is not important in the pathogenesis of rhabdomyosarcoma.

Epithelioid rhabdomyosarcoma: A rare case report mimicking carcinoma breast

Rhabdomyosarcoma (RMS) is an unusual sarcoma in adults. There are four main types of RMS described in the literature, including embryonal, alveolar, pleomorphic, and spindle cell type. Epithelioid RMS is a relatively newer form of RMS described in the literature, with little information regarding the natural course and management. We present a rare case of a young woman with metastatic epithelioid RMS, with an atypical presentation with bilateral large breast lumps, large ovarian mass progressing rapidly to metastasize, involving multiple sites. It has an aggressive clinical course with poor response to therapy.

Leiomyosarcoma of the urinary bladder: a case report

OBJECTIVES: To describe a case of urinary bladder leiomyosarcoma (LMS), a rare malignant mesenchymal tumor. Less than 1% of primary bladder tumors are LMS, and only 200 cases have been reported in the literature, since the first description by Gusshaver, a century ago.METHODS: A radical cystoprostatectomy, using Bricker reconstruction and rectosigmoidectomy was performed. In the intraoperative period, an extensive and hypervascularized mass was found, involving the abdominal wall and compressing the ureters and iliac arteries bilaterally. Resection of the extensive bladder and prostate lesion was permormed in monobloc. The resected material was sent to anatomopathological analysis, which characterized a pleomorphic sarcoma fusiform cell type with necrosis, measuring 30x25x13 cm. After immunohistochemistry, a grade I (stage II in the TNM classification) leiomyosarcoma was evidenced.RESULTS: The patient remained hospitalized for 15 days in the Intensive Care Unit (ICU) after acute renal failure after surgery, requiring dialysis. He had a good evolution in the postoperative period, despite maintaining dialysis for chronic renal failure. On the 22nd postoperative day, he was constipated, but with a functioning Bricker and with no particularities in the surgical wound.CONCLUSION: Although the therapeutic approach to bladder LMS is not a consensus in the literature, a more radical treatment can be justified in the situation of an aggressive tumor, which often has a poor prognosis.

Pouch of Douglas Liposarcoma—A Rarity

Introduction: This case report is one of a fairly common tumour in an extremely uncommon anatomic location. Statistically liposarcoma is the commonest type of soft issue malignancy, but publications of such a tumour arising from the pouch of Douglas (POD) to involve the uterus, are very few and far between. Case details: A 52-year-old woman presented with a mass in the lower abdomen, post-menopausal vaginal bleeding, and lower abdominal discomfort. Investigations revealed a large pelvic tumour that was attached to the posterior wall of a bulky uterus. There was no evidence of dissemination of the tumour to distant sites, and a laparotomy was performed. A massive soft tissue tumour occupied the POD. The tumour was dissected out from the surrounding structures, and the uterus and its appendages were removed in to. The histopathological examination revealed a liposarcoma of the pleomorphic type which was arising from the pouch of Douglas (POD), and was attached to the posterior wall of the uterus. Extensive leiomyomatous changes were seen in the uterus. Immunohistochemistry confirmed the liposarcoma to be of pleomorphic type. Conclusion: This case report is being published for its rarity and to illuminate the specific issues in the treatment of this ubiquitous tumour in an unusual site. The involvement of a Multidisciplinary Team (MDT) helps to choose the optimal combination of cytoreductive surgery, chemotherapy, and radiation for a given case with a POD malignancy.