Large Tumor Cells Research Articles

Breast Implant-Associated Anaplastic Large Cell Lymphoma: A Case Report

Breast implant-associated anaplastic large cell lymphoma (ALCL) is a recently recognized type of T-cell lymphoma that can develop following breast implants, with morphologic and immunophenotypic features indistinguishable from those of ALK-negative ALCL. Here we report a case of a 58-year-old woman with a history of subglandular silicone implants placed for bilateral breast augmentation 25 years ago, who presented with bilateral breast pain and was found to have bilateral Baker Grade III capsular contracture, and heterogenous fluid collection centered near the left third costochondral articulation, a suspicious left chest wall lesion, and left axillary lymphadenopathy on imaging. A left axillary lymph node core biopsy and an aspiration of the fluid were performed, and no malignant cells were identified. The patient underwent bilateral removal of breast implants and total capsulectomies. Microscopic examination of the capsule surrounding the left breast implant revealed large pleomorphic tumor cells in a fibrinous exudate. By immunohistochemistry, the tumor cells were found to be positive for CD3 (subset), CD4, CD7, CD30 (strong and uniform), and CD43, and negative for CD2, CD5, CD8, and ALK1, supporting the diagnosis of breast implant-associated ALCL. No lymphoma cells were identified in the right breast capsule, confirmed by CD30 stain. Breast implant-associated ALCL is a very rare disease that can develop many years after breast implant placement. Proper evaluation with breast imaging and pathologic workup is essential to confirm the diagnosis in suspected cases. Our case highlights that adequate sampling is important in the investigation of patients with suspected breast implant-associated ALCL.

Gamma Secretase Inhibitor: Therapeutic Target via NOTCH Signaling in T Cell Acute Lymphoblastic Leukemia.

T-cell acute lymphoblastic leukemia (T-ALL) is a diseased condition of bone marrow and lymphoblast, mainly expressed on T-cell immune phenotype. Diagnosis of TALL patients shows the burden of a large tumour and leukemia cells in the peripheral blood vessel which often infiltrates into the central nervous system. Chemotherapy is considered the primary mode of treatment for this disease, but recent advancements in the molecular understanding of the disease, including NOTCH1 signaling, could offer some alternatives. NOTCH signaling undergoes a non-regulated mutation at NOTCH1 in most T-ALL. Gamma-secretase (GS) plays a key role in blocking of proteolytic activation of NOTCH receptors, which could potentially be a new targeted therapy for this type of leukaemia. This study mainly aims to outline the role of γ-secretase inhibitors via NOTCH signaling in TALL. The role of GSI (γ-secretase inhibitor) in most T-ALL cell lines has been linked to the targeting pathway of NOTCH signaling. Mutation at NOTCH1 has however not served as a predictor of γ-secretase inhibitor sensitivity because of several factors, including gene expression of NOTCH pathway activity. Therefore, despite the promising outcome of this approach in NOTCH-1 activated T-ALL, not all patients with this condition are expected to respond. Long-term therapeutic success against cancerous cells is rarely achieved with monotherapy, and even targeting investigational pathways such as NOTCH may require a combination regimen. Ultimately, the optimised use of these new therapeutic agents may become the next tool in our search for an effective 'individualized medicine'.

Metastasis in the mandible involving gingiva

Oral metastasis, although rare, tends to involve jawbones, particularly the posterior region of the mandible, and involvement of oral soft tissues, even when less likely, is most often seen on the gingiva and tongue. Clinically, the soft-tissue masses tend to mimic pyogenic granuloma, peripheral giant cell granuloma or an epulis and thus are difficult to diagnose and identify. The jaw bone is preferred by prostate carcinoma as a metastatic target. Prostate malignancy, which is more common in Western countries than in India, may be adenocarcinomas or carcinomas. Oftentimes, metastatic lesions develop in the alveolar region and are a cause for tooth mobility, yet, they tend to be detected only after extraction of the affected tooth. In such cases, the symptomatic presentation therefore, is vague and indicative of tooth mobility secondary to periodontal pathology unless, a detailed history and follow-up is done. We report a case of a male patient who presented to our department with a proliferative, painful, swelling postextraction of the left first molar region, and the lesion was seen at the extraction site as well as in the mandibular anterior tooth region. The swelling was associated with palpable lymph nodes. Orthopantomogram showed an irregular, radiolucent lesion extending from the lower left central incisor to the left first molar region in the mandibular alveolus. Incisional biopsy tissue came with provisional diagnosis of osteomyelitis or squamous cell carcinoma as the patient was a habitual bidi smoker for more than 20 years. Histologically, it was an undifferentiated tumor with tumor cells seen in deep connective tissue with a lack of lineage differentiation. An undifferentiated malignant tumor represents either a metastasis of unknown origin or a primary neoplasia without obvious cell line of differentiation. Immunohistochemistry (IHC) of undifferentiated tumors helps to categorize them into small round blue cell tumors or large cell tumors. The oral pathologist was perplexed as there was no mention of any other malignancy in the patient's history, which, however, was noted by the surgeons few days later. Hence, initially, a hematopoietic malignancy was suspected which was ruled out by IHC, and later, staining with cytokeratin 7 (CK7), CK-high molecular weight and P63 confirmed prostate metastases as all three were negative.

A rare case of gastric outlet obstruction due to a large osteoclast-like giant cell tumor of the pancreatic head - A case report

A rare case of gastric outlet obstruction due to a large osteoclast-like giant cell tumor of the pancreatic head - A case report

The Clinicopathological Features of Epithelioid Undifferentiated Sarcoma with TFE3 Amplification with One Case Report

Herein, we first reported a case of undifferentiated sarcoma with epithelioid features harboring TFE3 amplification. A 66-year-old woman with a history of chronic lymphocytic leukemia and chemotherapy presented with a 4 cm palpable nodule in the left lower leg. Microscopically, the large epithelioid tumor cells with remarkable pleomorphism and the small round tumor cells intermingled with each other in a diffuse sheet or a hemangiopericytoma-like vascular growth pattern. Atypical mitotic figures and lymph node metastasis were found while tumor necrosis was absent. Immunohistochemically, the tumor was positive for vimentin, TFE3, and CD34. TFE3 gene amplification was identified by fluorescence in situ hybridization. The patient was alive and well without recurrence or metastasis for 12 months after tumor resection. The tumor should differentiate from PEComa, alveolar soft part sarcoma, epithelioid sarcoma, epithelioid angiosarcoma, and epithelioid rhabdomyosarcoma.

Primary cutaneous peripheral T-cell lymphoma, not otherwise specified: results of a multicentre European Organization for Research and Treatment of Cancer (EORTC) cutaneous lymphoma taskforce study on the clinico-pathological and prognostic features.

Cutaneous peripheral T-cell lymphoma, not otherwise specified (PTL NOS) is an aggressive, but poorly characterized neoplasm. The European Organization for Research and Treatment of Cancer cutaneous lymphoma taskforce (EORTC CLTF) investigated 33 biopsies of 30 patients with primary cutaneous PTL NOS to analyse their clinical, histological, immunophenotypic features and outcome. Retrospective analysis of clinical data and histopathological features by an expert panel. Cutaneous PTL NOS manifested clinically either with solitary or disseminated rapidly grown ulcerated tumours or disseminated papulo-nodular lesions. Histologically, a mostly diffuse or nodular infiltrate in the dermis and often extending into the subcutis was found. Epidermotropism was rarely present and only mild and focal. Unusual phenotypes were frequent, e.g. CD3+ /CD4- /CD8- and CD3+ /CD4+ /CD8+ . Moreover, 18% of the cases exhibited an aberrant expression of the B-cell marker CD20 by the tumour cells. All solitary tumours were located on the limbs and presented a high expression of GATA-3 but this did not correlate with outcome and therefore could not serve as a prognostic factor. The prognosis was shown to be generally poor with 10 of 30 patients (33%) dying of lymphoma within the follow-up of 36months (mean value; range 3-144). The survival rates were 61% after 3years (CI, 43-85%) and 54% after 5years (CI, 36-81%). Small to medium-sized morphology of tumour cells was associated with a better outcome than medium to large or large tumour cells. Age, gender, clinical stage, CD4/CD8 phenotype and GATA-3 expression were not associated with prognosis. Chemotherapy was the most common treatment modality, but surgical excision and/or radiotherapy may represent an appropriate first-line treatment for solitary lesions. Cutaneous PTL NOS shows an aggressive course in most patients independent of initial presentation, age and phenotype. Cytomorphology was identified as a prognostic factor. The data indicate a need for more effective treatment modalities in PTL NOS.

Evaluation of conditional treatment effects of adjuvant treatments on patients with synovial sarcoma using Bayesian subgroup analysis

BackgroundThe impact of adjuvant chemotherapy or radiation therapy on the survival of patients with synovial sarcoma (SS), which is a rare soft-tissue sarcoma, remains controversial. Bayesian statistical approaches and propensity score matching can be employed to infer treatment effects using observational data. Thus, this study aimed to identify the individual treatment effects of adjuvant therapies on the overall survival of SS patients and recognize subgroups of patients who can benefit from specific treatments using Bayesian subgroup analyses.MethodsWe analyzed data from patients with SS obtained from the surveillance, epidemiology, and end results (SEER) public database. These data were collected between 1984 and 2014. The treatment effects of chemotherapy and radiation therapy on overall survival were evaluated using propensity score matching. Subgroups that could benefit from radiation therapy or chemotherapy were identified using Bayesian subgroup analyses.ResultsBased on a stratified Kaplan–Meier curve, chemotherapy exhibited a positive average causal effect on survival in patients with SS, whereas radiation therapy did not. The optimal subgroup for chemotherapy includes the following covariates: older than 20 years, male, large tumor (longest diameter > 5 cm), advanced stage (SEER 3), extremity location, and spindle cell type. The optimal subgroup for radiation therapy includes the following covariates: older than 20 years, male, large tumor (longest diameter > 5 cm), early stage (SEER 1), extremity location, and biphasic type.ConclusionIn this study, we identified high-risk patients whose variables include age (age > 20 years), gender, tumor size, tumor location, and poor prognosis without adjuvant treatment. Radiation therapy should be considered in the early stages for high-risk patients with biphasic types. Conversely, chemotherapy should be considered for late-stage high-risk SS patients with spindle cell types.

Collision tumour of two nodal metastases (adenocarcinoma and mast cell tumour) in a dog

An 11-year-old female neutered Labrador retriever was referred for a large vulvar mast cell tumour that extended into the vagina. Abdominal ultrasound revealed an enlarged right medial iliac lymph node, and cytology of the node was consistent with an endocrine/neuroendocrine tumour. An approximately 1.5-cm right anal sac mass was palpated. Medial iliac lymphadenectomy via ventral celiotomy, right anal sacculectomy and marginal vulvar mass resection were performed. Histopathology was consistent with right anal sac adenocarcinoma, vulvar mast cell tumour, and the right medial iliac lymph node showed a metastatic collision of both the adenocarcinoma and mast cell tumour cell populations. Adjunctive chemotherapy and radiotherapy were recommended to address the risk of local recurrence and further metastasis but were declined by the owner. The dog remains alive and asymptomatic with no visible evidence of recurrence 14 months after initial presentation.

Cytology of SMARCA4-Deficient Thoracic Neoplasms: Comparative Analysis of SMARCA4-Deficient Non-Small Cell Lung Carcinomas and SMARCA4-Deficient Thoracic Sarcomas

Introduction: Inactivating mutations of the SMARCA4 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily A, member 4) gene and/or loss of the BRG1 (brahma-related gene 1) protein defines SMARCA4-deficient thoracic sarcoma (SMARCA4-dTS), an aggressive neoplasm with a usually fatal outcome. Similar SMARCA4 mutations/BRG1 loss is also seen in a subset of non-small cell lung carcinomas (NSCLCs; SMARCA4-dNSCLCs) that lack alterations in currently targetable oncogenic drivers, that is, EGFR, ALK, and ROS1. There is limited knowledge on the cytomorphological features of these SMARCA4-deficient thoracic neoplasms. Methods: We retrospectively analysed the cytology of 2 cases each of SMARCA4-dNSCLC and SMARCA4-dTS to understand their cytomorphological overlap, if any, and identify features that would prompt testing for BRG1 loss. Results: All 4 patients were males presenting with advanced disease, with a mean age of 41.5 years (SMARCA4-dTS) and 58.5 years (SMARCA4-dNSCLC) at presentation. The cytology of the 2 SMARCA4-dTSs was strikingly similar, showing predominantly singly dispersed rhabdoid phenotype tumour cells with perinuclear cytoplasmic condensations in an inflammatory or necrotic background. The cytology raised suspicion for a wide range of differentials, including melanoma, high-grade lymphoma, germ cell tumour, undifferentiated carcinoma, and undifferentiated sarcoma. SMARCA4-dNSCLCs, on the other hand, were recognizable as poorly differentiated (adeno)carcinomas and were easily distinguished from SMARCA4-dTSs, with both cases showing cohesive clusters of frequently large tumour cells with abundant pale cytoplasm. Conclusion: A diagnosis of SMARCA4-dTS is possible on cytology with appropriate ancillary testing and a high index of suspicion. The cytology of SMARCA4-dNSCLCs does not overlap with SMARCA4-dTS; rather, it resembles that of any poorly differentiated (adeno)carcinoma in the limited numbers analysed in this study.

Transfert osseux libre de condyle fémoral médial pour la reconstruction d’une perte de substance osseuse carpo-métacarpienne après résection d’une tumeur à cellule géante des gaines synoviale

We report the case of a 23-year-old patient treated for a large giant cell tumour of the synovial sheaths of the ulnar edge of the hand and wrist invading the triquetrum, the hamatum, the ulnar part of the capitate as well as the bases of the 3rd, 4th and 5th metacarpals and the floor of the Guyon's canal and the carpal tunnel. A monobloc resection was performed in a healthy margin; the loss of bone substance was 7×3.5cm. We performed a bone reconstruction using a cortico-cancellous medial femoral condyle free flap of 8×4cm. Postoperative follow-up was uneventful and bone consolidation was achieved at 2,5 months postoperatively. The cortico-cancellous medial femoral condyle free flap is an interesting option for treating small and medium size bone loss in the hand and wrist. Its use in its pure bone form or in its chimeric form with muscle, cartilage or skin opens up a wide range of choices for the reconstructive surgeon.

Abstract 6218: Discovery and preclinical evaluation of AMG 397, a potent, selective and orally bioavailable MCL1 inhibitor

Abstract MCL1 inhibitors are an emerging class of therapeutics targeting key protein-protein interactions between MCL1 and BH3 domain containing pro-apoptotic BCL-2 family members. Several MCL1 inhibitors have entered clinical trials including AMG 176, currently in Phase I clinical development for hematologic malignancies. However, all existing clinical stage MCL1 inhibitors are being administered intravenously. Here we describe the discovery and preclinical evaluation of AMG 397, the first orally administered MCL1 inhibitor in clinical development. The pursuit of an orally bioavailable MCL1 inhibitor with an acceptable human clinical dose required improvements to AMG 176 in both potency and pharmacokinetic properties. The chemistry effort to identify AMG 397 began with the observation that there were two conformations in AMG 176 bound to MCL1 with a difference of 2.8 kcal/mol in energy. A structure-guided approach in combination with ligand-based design to attempt to favor the more active conformation provided the advanced lead AM-3106 (Ki=100 pM) with significantly improved cell based potency (OPM2 viability IC50 = 19 nM) compared to AMG 176. Further refinements in potency and PK properties of AM-3106 culminated in the discovery of AMG 397. AMG 397 exhibited picomolar affinities for MCL1 (Ki=15 pM), selectively competing for binding to the BH3-binding groove of MCL1 with pro-apoptotic BCL-2 family members (e.g. BIM). In cells, AMG 397 potently disrupted the interaction between MCL1 and BIM. Treatment of OPM2 cells, an MCL1 dependent multiple myeloma cell line, induced clear increases in Caspase-3/7 activity within one hour. Subsequent effects on viability were observed across a treatment time course with maximal effects observed following 24 hours of continuous exposure (IC50=50 nM). Time course washout viability studies demonstrated that AMG 397 induced a committed step towards apoptosis and suggested that discontinuous dosing schedules would be sufficient to achieve antitumor effects in vivo. Cell lines from hematologic malignancies including AML, multiple myeloma and DLBCL exhibited greatest sensitivity to AMG 397 in a large tumor cell line profiling screen (n=300). In vivo, oral administration of AMG 397 demonstrated rapid and sustained dose dependent increases in activated BAK, cleaved Caspase-3 and cleaved PARP in established OPM2 xenografts. Discontinuous dosing schedules of once or twice weekly at 25 or 50 mg/kg of AMG 397 exhibited significant tumor regressions in mice bearing OPM2 xenografts, with 9 of 10 mice tumor free at the end of the study in both 50 mg/kg groups. AMG 397 was also tested in the MOLM-13 orthotopic model of AML where twice weekly administration at 10, 30 and 60 mg/kg achieved 47% tumor growth inhibition (TGI), 99% TGI and 75% regression respectively. Combination of AMG 397 at 10 mg/kg twice weekly and 50 mg/kg of venetoclax daily achieved 45% regression in the same model. Citation Format: Sean Caenepeel, Rex Karen, Brian Belmontes, Alla Verlinsky, Hong Tan, Yajing Yang, Xiaoyue Chen, Kexue Li, Jennifer Allen, Jan Wahlstrom, Jude Canon, Angela Coxon, Paul Hughes. Discovery and preclinical evaluation of AMG 397, a potent, selective and orally bioavailable MCL1 inhibitor [abstract]. In: Proceedings of the Annual Meeting of the American Association for Cancer Research 2020; 2020 Apr 27-28 and Jun 22-24. Philadelphia (PA): AACR; Cancer Res 2020;80(16 Suppl):Abstract nr 6218.

Abstract 1107: Cell state diversity promotes metastasis through heterotypic cluster formation in melanoma

Abstract Metastasis is the primary cause of cancer death, yet we know little about what determines its timing and aggressiveness. In melanoma, transcriptional profiling has revealed multiple co-existing cell states, including proliferative (PRO) versus invasive (INV) subpopulations that have been posited to represent a “go or grow” tradeoff in the etiology of metastasis. Although both of these populations are present in primary tumors and metastases, little is known about how they physically interact with each other in tumor dissemination. We use a combination of melanoma modeling in zebrafish and human validation to demonstrate that these subpopulations form spatially structured heterotypic clusters that cooperate in the seeding of metastasis. We unexpectedly found that INV cells were tightly adherent to each other and formed large tumor cell clusters. Mixing of PRO and INV cells generated heterotypic clusters, composed of a dense central core of INV cells surrounded by a rim of PRO cells. Using intravital imaging, we identified metastatic cooperation between these two subpopulations, in which the INV cells facilitated the earlier spread of the less metastatic PRO cells via collective extravasation. Mechanistically, we identified the TFAP2 family of neural crest transcription factors to be a master regulator of both clustering and the PRO/INV states. Knockout of TFAP2 from melanoma cells reduced proliferation but increased clustering and metastasis. TFAP2 loss resulted in increased expression of cell adhesion molecules, providing a mechanistic basis for cluster formation. Our data suggest a coherent framework for the co-existence of these two divergent cell populations in melanoma, in which differing cell states form heterotypic clusters that promote collective metastasis via cell-cell cooperation. Citation Format: Nathaniel R. Campbell, Maomao Zhang, Maayan Baron, Silja Heilmann, Maxime Deforet, Lorenza Ferretti, Ting-Hsiang Huang, Mitchell P. Levesque, Itai Yanai, Joao B. Xavier, Richard M. White. Cell state diversity promotes metastasis through heterotypic cluster formation in melanoma [abstract]. In: Proceedings of the Annual Meeting of the American Association for Cancer Research 2020; 2020 Apr 27-28 and Jun 22-24. Philadelphia (PA): AACR; Cancer Res 2020;80(16 Suppl):Abstract nr 1107.

Cyto-histo correlations of plasmacytoid and micropapillary variants of high-grade urothelial carcinoma: do they fit well in The Paris System for reporting urinary cytology?

Plasmacytoid and micropapillary variants of high-grade urothelial carcinoma (HGUC) exhibit unique histologic morphology and very aggressive clinical behavior. However, the morphology of these 2 variants in urinary cytology is not well studied and evaluated using The Paris System for reporting urinary cytology. A database search was performed in all patients with the diagnosis of plasmacytoid or micropapillary HGUC. A total of 5 patients with positive urinary cytology cases were identified. The cytomorphology of every urinary cytology case was correlated with the histologic features in the surgical specimens from the same patient. One urine and 4 bladder washings were evaluated. Cytologically, plasmacytoid HGUCs are characterized by single, large tumor cells with hyperchromasia, irregular nuclear membranes, and vacuolated cytoplasm. The nuclear-to-cytoplasmic (N:C) ratio was less than 0.5 in many of the malignant cells due to the abundant cytoplasm. The cytology features of micropapillary HGUC include the presence of micropapillae of tumor cells with no fibrovascular core. Individual high-grade urothelial cells were also identified in all 4 cases, but 1 (25%) of these had only rare cells meeting The Paris System criteria for HGUC due to abundant cytoplasm and lack of hyperchromasia in most malignant cells. Plasmacytoid and micropapillary variants of HGUC have unique cytomorphologic features in urinary cytology specimens, which are reflective of the corresponding histological findings. These 2 clinically aggressive variants of HGUC may not be as readily interpreted as malignant using The Paris System for reporting urinary cytology, creating potential diagnostic pitfalls.

SUN-940 Carney Complex: A Case of a Rare Multiple Endocrine Neoplasia Misdiagnosed as Peutz-Jeghers Syndrome

Carney Complex (CNC) is an extremely rare multiple endocrine neoplasia caused by germline inactivating mutation in protein kinase A type I-alpha regulatory subunit (PRKAR1A gene). Mode of inheritance is mostly autosomal dominant; 25% of cases are due to de novo mutations. Only 750 world-wide cases have been reported. Most patients are diagnosed in the second or third decade. Clinical features include cutaneous myxomas, angiomyxoid nodules, lentiginous skin pigmentation, cardiac myxomas, and benign and rare malignant endocrine tumors. These endocrine tumors include and are not limited to prolactinomas, thyroid tumors, primary pigmented nodular adrenocortical disease (PPNAD), and large cell-calcifying Sertoli cell tumors (LCCSCT). Diagnosis is often challenging as disease manifestations can occur sporadically over a large span of time, and patients may present with various conditions such as Cushing syndrome, like our case. We present a case that demonstrates the importance of early recognition of this rare disorder.A 28-year-old Caucasian male with PMH of HFrEF, HTN, Sertoli cell tumor status post orchiectomy, vertebral fractures, and surgical removal of lip angiomyxoma presented to clinic for hypogonadism. Physical examination revealed marked Cushingoid features and facial lentigines above his eyes and on his lips. His eclectic medical history and unique exam findings lead to finding of a unifying diagnosis. His labs revealed severe Cushing syndrome, and computed tomography (CT) of his abdomen was performed due to ACTH independent hypercortisolism, demonstrating a bilateral lobular appearance of the adrenal glands.Combination of labs and physical exam findings of lentigines, skin myxomas, cushingoid features, rare angiomyxoma, LCCSCT and hypercortisolism lead to diagnosis of Carney Complex. He was misdiagnosed with Peutz-Jeghers in his adolescence due to LCCSCT and mucosal lentigines; therefore, hormonal screening was not routinely performed. Untreated Cushings led to severe osteoporosis with vertebral fractures and heart failure. Treatment included bilateral adrenalectomy. Pathology report confirmed rare PPNAD. PPNAD and LCCSCT are extremely rare tumors almost exclusively linked to Carney Complex. Interestingly, family history did not reveal endocrine disorders, cancers, or severe illnesses. Genetic testing returned positive for the PRKAR1A gene mutation. Given the consequences of untreated hormonal aberrations seen in this disorder, an early and accurate diagnosis is imperative.

Pediatric granular cell tumor in the posterior wall of the larynx extending to the trachea.

Granular cell tumor (GCT) is a slow-growing benign neoplasm that can be found in any organ. Pediatric laryngotracheal GCT is rare. We experienced a 6-year-old boy suffering from a barking cough and symptoms of stridor and croup for one month. Head and neck computed tomography revealed a protruding mass that occluded 60% of the airway lumen. Under the impression of hemangioma or papilloma, excision revealed a submucosal non-encapsulated mass. Histologically, the mass was composed of sheets of large polyhedralshaped tumor cells containing plump eosinophilic granular cytoplasm and centrally placed, small, bland-appearing nuclei. The tumor cells were positive for S-100 protein, and voluminous eosinophilic cytoplasm was stained by diastase-resistant periodic acid-Schiff. The present report describes a unique case of a huge pediatric laryngeal GCT extending to the subglottic trachea. We also review the clinical course of pediatric laryngotracheal GCT and emphasize the importance of diagnosing GCT in children.

Thoracoscopic surgery approach to mediastinal mature teratomas: a single-center experience

BackgroundMediastinal mature teratomas are rare tumors with diverse surgical approaches. The aim of this study is to review our experience of thoracoscopic surgery management in patients with teratomas.MethodsWe retrospectively reviewed 28 consecutive patients with mediastinal mature teratomas who underwent thoracoscopic surgery at Viet Duc University Hospital from January 2008 to August2018. Patients were divided into 2 groups with 2 types of thoracoscopic surgery, closed thoracoscopic surgery (CTS) group and video-assisted thoracoscopic surgery (VATS) group. The selection of sugical approach was based on sizes, locations and characteristics of tumors. Post-operative outcomes were assessed and compared between these 2 groups.ResultsThere were 14 female and 14 male patients with a median age of 41.2 ± 13.8 years. A total of 22 teratomas were located on the right side of the chest cavity and 6 on the left side. We performed CTS in 21 patients (75%) and VATS in 7 patients (25%) for tumor resection. There were 3 cases (10.7%) required conversion to minithoracotomy (5 cm in incision length). Skin appendages accounted for the highest rate (96.4%) in pathology. There was no record of mortality or tumor recurrence detected by computerized tomography.ConclusionA thoracoscopic surgery for a mediastinal mature teratoma was a feasible choice. Challenging factors such as large tumors, intraoperative bleeding and strong tumor cell adhesion were considered handling by conversion to mini-thoracotomy that could ensure safety procedures and complete removal of tumors. Extraction of tumor contents might be performed for patients with large mature cystic teratomas to facilitate thoracoscopic surgery.

Diagnostic utility of programmed cell death ligand 1 (clone SP142) in mediastinal composite lymphoma: A report of two cases

Composite lymphoma is a well-known diagnostic entity exhibiting the synchronous occurrence of two or more distinct types of lymphomas in the same specimen. Here we report two patients, a 14-year-old female (Case 1) and a 45-year-old male (Case 2), with mediastinal composite lymphoma, comprising nodular sclerosis classic Hodgkin lymphoma (NSCHL) and primary mediastinal large B-cell lymphoma (PMBL). Both patients had a mediastinal mass, and manifested two different histologic components in the same biopsy, one characteristic of NSCHL and the other PMBL. The NSCHL areas included Hodgkin and Reed-Sternberg (HRS) cells with typical immunophenotypic features (CD30-positive and CD20-negative), whereas the sheets of large tumor cells characteristic of PMBL were strongly and uniformly CD20-positive. Interestingly, although both cases showed neoplastic PD-L1 (nPD-L1) positivity on the HRS cells of NSCHL, they differed regarding nPD-L1 expression on the PMBL tumor cells. In Case 1, the nPD-L1-negative PMBL component was anatomically situated outside the NSCHL lesion. On the other hand, in Case 2, the nPD-L1-positive PMBL component was characterized by transitional or continuous areas with the NSCHL component. These findings suggested that nPD-L1 expression may define two subtypes of PMBL that are more similar to or distinct from classic Hodgkin lymphoma.

Large giant cell tumour of distal femur in 88 year old male patient: A rare case report and review of literature

Giant cell tumour is a locally aggressive benign tumour of bone predominantly involving age group 20-40 years. We are reporting a case of giant cell tumour of distal femur in an 88 year old male which was treated with extended curettage and bone cement. At two years follow up the patient is disease free with no recurrence or metastasis and achieved 80 degree of knee flexion.

Large giant cell tumor of the posterior iliac bone – an atypical location. A case report and literature review

Giant cell tumor (GCT) is a locally aggressive tumor but with benignity features, representing approximately 18% of non-malignant bone tumors in European countries, with slight female predominance. Malignancy in GCT is rare, about <2% of cases and is more common at older ages. Is known that usually occurs at the epiphyses of long bones, but extremely rare may have another location, such as the pelvic bone. An atypical location – the posterior iliac bone, found at a 34-year-old male –, is the case report we studied and described. Starting from the patient’s complains, like a mass in the left buttock region described as “recently appeared”, firm, not-mobile, with no distinctive borders and no tenderness at palpation, and a recent history trauma, multiple investigations have been performed, which have highlighted an osteolytic lesion, close to the sacroiliac joint, only with infiltration of the gluteal, iliac and paravertebral muscles. The treatment of choice was hemi-pelvectomy, with wide tumoral resection, and selective embolization of the nutrient vessels 24 hours prior to the surgical procedure. At two years postoperative, we found a good functional result and the computed tomography (CT) scan revealed no signs of recurrence.

Clinicopathological significance of CD79a expression in classic Hodgkin lymphoma.

Classic Hodgkin lymphoma (CHL) is a lymphoid neoplasia characterized by the presence of large tumor cells, referred to as Hodgkin and Reed-Sternberg (HRS) cells, originating from B-cells in an inflammatory background. As the clinical significance of B-cell markers has yet to be fully elucidated, this study aimed to clarify the clinicopathological significance of CD79a in 55 patients with CHL. They were immunohistochemically divided into two groups, comprising of 20 CD79a-positive and 35 CD79a-negative patients. There was no significant correlation between CD79a and CD20 expression (rs = 0.125, P = 0.362). CD79a-positive patients were significantly older at onset (P = 0.011). There was no significant correlation between CD79a-positivity and clinical stage (P = 0.203), mediastinal involvement (P = 0.399), extranodal involvement (P = 0.749), or laboratory findings, including serum levels of lactate dehydrogenase (P = 1) and soluble interleukin-2 receptor (P = 0.251). There were significant differences in overall survival (OS) (P = 0.005) and progression-free survival (PFS) (P = 0.007) between CD79a-positive and CD79a-negative patients (5-year OS: 64.6% and 90.5%; 5-year PFS: 44.0% and 76.6%, respectively). Five patients in whom the majority (> 80%) of HRS cells expressed CD79a consisted of 4 males and 1 female aged between 52 and 81 years; 4 of them were in a limited clinical stage. We concluded that CD79a-positive CHL may have unique clinicopathological features.