Rare Aggressive Tumor Research Articles

Revealing the structural microenvironment of high metastatic risk uveal melanomas following decellularisation

Uveal melanoma (UM) is a rare aggressive intraocular tumour that spreads most commonly to the liver in tumours with loss of one copy of chromosome 3 (HR-M3); current treatments for metastatic disease remain largely ineffective. Pre-clinical research is increasingly using three-dimensional models that better recapitulate the tumour microenvironment (TME). One aspect of the TME is the acellular extracellular matrix (ECM) that influences cell proliferation, migration and response to therapy. Although commercial matrices are used in culture, the composition and biochemical properties may not be representative of the tumour ECM in vivo. This study identifies UM metastatic risk specific ECM proteins by developing methodology for decellularisation of low- and high- metastatic risk tissue samples (LR-D3 vs. HR-M3). Proteomic analysis revealed a matrisome signature of 34 core ECM and ECM-associated proteins upregulated in HR-M3 UM. Combining additional UM secretome and whole cell iTRAQ proteomic datasets revealed enriched GO and KEGG pathways including ‘regulating ECM binding’ and ‘PI3K/Akt signalling’. Structural analyses of decellularised matrices revealed microarchitecture of differing fibre density and expression differences in collagen 4, collagen 6A1 and nidogen 1, between metastatic risk groups. This approach is a powerful tool for the generation of ECM matrices relevant to high metastatic risk UM.

RECAPITULATING THE POST-SURGICAL BRAIN MICROENVIRONMENT OF ATYPICAL TERATOID RHABDOID TUMOURS TO IDENTIFY MEMBRANE PROTEINS FOR TARGETED THERAPY

Abstract AIMS Atypical Teratoid Rhabdoid Tumours (AT/RT) are rare aggressive tumours primarily arising in the hind brain of children under three, conferring median survival of <12 months. Surgery remains the only standard treatment; however tumours recur from post-surgical residual disease. Decellularised human brain cerebellum aim to recapitulate AT/RT post-surgical brain microenvironments which retain extracellular matrix (ECM) ligands, and where AT/RT plasma membrane proteins are hypothesised to represent viable molecular therapy targets. METHOD AT/RT, astrocyte and human brain cerebellum (decellularised and non-decellularised) membrane protein fractions were extracted and analysed using liquid chromatography-mass spectrometry. Proteomic analysis tools were integrated to identify differential expressed proteins and identify shared correlations. RESULTS Decellularised cerebellar ECM exhibited an enriched membrane proteome relative to total proteome, evidence of complete removal of intracellular components. Furthermore, decellularised and non-decellularised cerebellar tissue expressed comparable levels of membrane proteins and protein networks, indicative of ECM ligand retention. AT/RT and astrocyte KEGG Pathway analysis revealed specific oncology-based pathway expression in membrane protein compared to total protein, reinforcing membrane proteins as more visible therapeutic targets. Between the top 20 expressed proteins, ATP1A1 was the only membrane protein shared exclusively between all AT/RT cells and astrocytes, suggesting a critical functional role. Membrane proteins showing shared expression when normalised against each AT/RT cell line included ATP1A1, HSPD1, GNA13 and SLC3A2, indicating biological similarities between AT/RT molecular subtypes, which may offer candidate ubiquitous therapy targets. Membrane proteins expressed similarly in AT/RT-MYC molecular subtypes include ATP1A1, SLC7A5 and EEF1A1. STRING membrane pathway analysis also identified shared proteins between AT/RT and astrocyte cell populations. CONCLUSION AT/RT membrane protein pathways provide ideal therapeutic targets directly amenable for drug repurposing. Future work prioritises proteomic analysis utilising 3D AT/RT spheroids and cerebellum brain tissue to maximise accurately recapitulating the AT/RT post-surgical microenvironment. Cerebellum decellularisation still permits retention of essential proteins and ligands.

Poorly differentiated chordoma: recognising this complex and rare aggressive tumour with characteristic immunohistochemical profile.

Poorly differentiated chordoma (PDC) is an uncommon subtype of chordoma, distinct in its occurrence in paediatric age group, location, variable epithelioid/rhabdoid/spindled histomorphology and the lack of physaliphorous cells (classical of chordoma) and immunohistochemistry (INI-1 loss, brachyury positive). We describe two cases of PDC. A3-year-old male and 4-year-old female child presented with neck stiffness and infiltrating tumour involving the skull base and upper cervical vertebral segments. Histopathology showed a tumour with sheets of cells having epithelioid to rhabdoid morphology and absence of physaliphorous cells. The tumour cells were positive for pan-cytokeratin, EMA, CD99 and vimentin and showed loss of INI-1 suggesting differentials of epithelioid sarcoma and atypical teratoid/rhabdoid tumour. On careful review of the clinical, radiological and pathological features, the additional immunohistochemistry for brachyury was performed, and its positivity clinched the diagnosis of PDC. Both the patients succumbed within a short span post-surgery. The present case study helps in creating an awareness and attempts to expand our knowledge in relation to the spectrum of chordoma (clinico-histological) and its immunohistochemical profile.

Uterine carcinosarcomas: A case series of 9 cases from a low-income country.

Uterine carcinosarcomas (UCS) are rare aggressive biphasic tumors classified as a subtype of high-grade uterine carcinomas. However, these tumors have particular histopathological features and clinical behavior with worse prognosis than high-grade uterine carcinomas. The incidence of UCS is increasing and more studies are required to elucidate their clinical and histopathological characteristics. Herein, we report clinicopathological features of 9 cases of UCS in a low-income country. We retrospectively collected all cases of UCS at our Pathology Department over a period of 4 years. The diagnosis was performed on formalin-fixed, paraffin-embedded and hematoxylin and eosin-stained surgical specimens. Nine surgically treated cases of UCS have been registered, representing 12.67% of all uterine malignancies with a mean age of 58.88 years (range: 50-65 years). Abdominal pain and metrorrhagia were the main clinical presentations. The epithelial component of UCS was often a serous carcinoma (66.66%) and patients presented with large tumors (mean size of 9.24 cm, range of 5-19 cm), with advanced FIGO stages (stages III-IV) in 5/9 patients (55.55%). Follow-up data were available in 5/9 patients among which only 2 were alive 2 and 25 months after the surgical treatment (overall survival of 40%). UCS are rare and aggressive uterine tumors with very poor prognosis especially in low-income countries.

Analysis of miR-483-3p and miR-630 expression profile in pediatric adrenocortical tumors and the effect of their modulation on adrenal tumorigenesis in vitro

Pediatric adrenocortical tumors (ACT) are rare aggressive neoplasms with heterogeneous prognosis. MicroRNA (miRNA) signatures have been associated with cancer diagnosis, treatment response, and outcomes of several types of cancer. However, the role played by miRNAs in pediatric ACT has been poorly explored. In this study, we have evaluated the expression of miR-483-3p and miR-630 in 67 pediatric ACT and 19 non-neoplastic adrenal samples, the effects of the modulations of these miRNAs, and their relationship with the TGF-β pathway in the H295R and H295A cell lines. Deregulation of both miRNAs was related to survival and disease advanced stages and hence to patients’ prognosis. Moreover, modified miR-483-3p and miR-630 in vitro expression decreased cell viability and colony formation capacity, changed how some genes of the TGF-β pathway, such as TGFBR1, TGFBR2, and SMAD7, are expressed, and altered Smad3, pSmad3, Smad 2/3, N-cadherin, and Vimentin protein expression. Besides that, when inhibition of the TGF-β pathway was combined with miR-630 overexpression or miR-483-3p silencing, cell viability and colony formation capacity decreased, and protein expression in the TGF-β pathway changed. Together, the data indicate that both miRNAs participate in the TGF-β pathway and are therefore potential markers for predicting the prognosis of patients with pediatric ACT.

FDG PET/CT in Staging of α-Fetoprotein-Producing Hepatoid Adenocarcinoma of the Pancreas.

Hepatoid adenocarcinoma of the pancreas is a rare aggressive tumor with poor prognosis. We describe contrast-enhanced CT and FDG PET/CT findings in a case of pancreatic hepatoid adenocarcinoma with significantly elevated α-fetoprotein level presenting as acute pancreatitis. The primary pancreatic tumor diffusely involved the pancreas and showed heterogeneous enhancement mimicking acute necrotizing pancreatitis on contrast-enhanced CT. FDG PET/CT showed intense FDG uptake (SUVmax, 24.5) of the left supraclavicular and retroperitoneal lymph node metastases, suggesting FDG PET/CT may be useful for staging of this aggressive tumor.

FDG PET/CT in Staging and Response Evaluation of Primary Urothelial Carcinoma of the Urethra.

Primary urethral urothelial carcinoma is a rare aggressive tumor with a high propensity for local invasion and regional and distal metastases. We describe the usefulness of FDG PET/CT in management of a patient with primary urethral urothelial carcinoma. FDG PET/CT at initial staging showed FDG-avid primary tumor and lymph node metastasis of the left groin, and mild or no activity of the lung metastases due to small size. FDG PET/CT after 4 cycles of chemotherapy showed progression of the primary tumor and lung metastases, partial response of the left inguinal lymphadenopathy, and multiple new sites of FDG-avid metastases.

Ameloblatoma: An aggressive odontogenic neoplasm with altered vickers and gorlin criteria

Plexiform ameloblastoma is a rare aggressive odontogenic neoplasm comprising about 1% of all odontogenic lesions of the jaw arising from cells of enamel organ that has failed to differentiate to form the hard tissues of tooth. Clinically, Ameloblastoma presents as painless swelling of jaw occurring in 3 and 4 decade in the posterior region of the mandible with equal sex distribution. It shows multiple patterns including solid, unicystic, extra osseous and desmoplastic type. Due to its varied presentation and relative risk of recurrence, it possesses a great challenge to physician for early diagnosis. Vickers and Gorlin (V and G) criteria is an integral part of diagnosis of histological types of Ameloblastoma. This article aims to highlight the variations observed in V and G criteria in the histopathological features of mixed variety of Ameloblastoma in a middle aged man.

Radiation-Induced Breast Angiosarcoma Associated with Homolateral Axillary Recurrence: A Case Report

Breast angiosarcoma is a rare aggressive tumor that develops from connective tissue of vascular origin with a tendency towards rapid recurrence and the appearance of visceral metastases. It occurs either as a primary tumor in young women or as a secondary tumor following breast irradiation. We report a case of breast angiosarcoma associated with homolateral axillary recurrence in a 45-year-old woman treated for bilateral breast cancer.

Pulmonary artery sarcoma affecting the pulmonary valve mistaken as pulmonary vasculitis: a case report and comparative literature review

Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.

18F-fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and 68Ga-prostate-specific Membrane Antigen Positron Emission Tomography/Computed Tomography Imaging in the Evaluation of Rare Entity Adult Embryonal Rhabdomyosarcoma of Prostate.

A 21-year-old male with embryonal rhabdomyosarcoma of the prostate was referred for 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) and 68Ga-prostate-specific membrane antigen (PSMA) PET/CT for initial disease staging. The PET scans revealed hypermetabolic and PSMA expressing lobulated mass involving both lobes of the prostate and weakly metabolic and PSMA expressing few bilateral pararectal and external iliac nodes, multiple bilateral lung nodules scattered over the lung parenchyma and multiple bone marrow lesions in both axial and appendicular skeleton. Magnetic resonance imaging prostate showed gross prostatomegaly with large lobulated T2 hyperintense heterogeneously enhancing mass lesion showing restricted diffusion, involving both lobes of the prostate with extraprostatic spread along anterior, posterior, and left lateral margins with evidence of lymph nodal and osseous metastases. The demonstration of increased uptake of 18F-FDG and 68Ga-PSMA in the primary as well as bilateral pararectal and external iliac nodes, multiple bilateral lung nodules, and multiple bone marrow lesions in both axial and appendicular skeleton indicates a potential role of 18F-FDG PET/CT and 68Ga-PSMA PET/CT in disease staging in this rare aggressive tumor of the prostate.

Teratocarcinosarcoma: A rare malignant tumor of sinonasal tract

Sinonasal teratocarcinosarcoma is an extremely rare malignant tumor arising in the sinonasal tract, having combined histological features of teratoma and carcinosarcoma. Here, we are presenting a case of sinonasal teratocarcinosarcoma in a 44 year‑old male patient. 44 year old male presented to us with complains of nasal obstruction and intermittent epistaxis since 1 month. On examination polypoidal mass was noted in left nasal cavity which bleeds on touch. On MRI, well defined heterogeneously enhancing lesion completely filling the left nasal cavity was noted. Histopathological examination of resected specimen revealed, malignant epithelial components being adenocarcinoma and squamous cell carcinoma, mesenchymal component composed of spindle cells, along with primitive mesenchymal and blastemal components. Immunohistochemistry showed epithelial and stromal markers positive for Cytokeratin and Vimentin respectively. Sinonasal teratocarcinosarcoma (TCS) is a rare and aggressive malignant neoplasm characterized by the combination of malignant teratoma and carcinosarcoma. Most common presentation being nasal obstruction and epistaxis with male preponderance. The tumor shows heterogeneous morphology with varying proportions of benign and malignant epithelial, mesenchymal, and blastemal components. Less than 100 cases have been reported in literature. Teratocarcinosarcoma is a rare aggressive sinonasal tumor. Total excision of the tumor and aggressive sampling for histopathological examination is necessary to avoid misdiagnosis of olfactory neuroblastoma and carcinosarcoma.

Extraskeletal Osteosarcoma with Extensive Gastrointestinal Tract Involvement

Abstract Introduction/Objective Extraskeletal osteosarcoma (ESOS) is a rare aggressive mesenchymal tumor that originates from soft tissues or visceral organs, differentiating it from primary bone osteosarcoma. ESOS represents less than 5% of all osteosarcomas and less than 1% of soft tissue sarcomas. Primary ESOS in the gastrointestinal tract (GIT) is exceedingly uncommon, with a low incidence rate Methods/Case Report A 70 year-old-man with a history of hypertension, Myasthenia Gravis, hypothyroidism (status post total thyroidectomy), and skin cancer presented to the clinic with a chief complaint of abdominal fullness of 4 months duration. Primary work-up revealed anemia (Hemoglobin 7 g/dl) and peripheral blood smear was suggestive of bone marrow infiltration. Evaluation with upper gastrointestinal endoscopy and ultrasound demonstrated regular mucosal folds of the stomach with a solid-cystic lesion between the body of the pancreas and the posterior aspect of the stomach. The patient underwent a fine needle biopsy showing a spindle cell lesion with positive immunohistochemistry (IHC) for Smooth Muscle Actin (SMA), beta-Catenin, CD34, and negative for panCK, DOG-1, and CD117; suggestive of leiomyoma. The patient underwent a total gastrectomy, distal pancreatectomy, and splenectomy. Examination of the specimen confirmed a high-grade malignant spindle cell lesion with numerous associated osteoclast-like multinucleated giant cells, and focal areas consistent with osteoid changes. The IHC was requested and the cells were positive for WT1 and Muscle Specific Actin, SATB2, and Ki-67>90% whereas the cells were negative for SMA, Myo-D1, Myogenin, S100, SOX10, MART1, CD34, CD31, and Calretinin. Also, the genetic analysis for MDM2 was negative. These findings confirmed the diagnosis of ESOS with abdominal visceral involvement (stomach, pancreas, and omentum) Results (if a Case Study enter NA) NA Conclusion ESOS is an extremely rare condition that demands a comprehensive evaluation involving clinical, radiological, histopathological, and immunohistochemical analyses to establish an accurate diagnosis. Distinguishing ESOS from primary bone osteosarcoma is vital due to the different treatment strategies. Additionally, ESOS requires a unique therapeutic approach compared to more prevalent gastrointestinal sarcomas like GIST or leiomyosarcoma. The mainstay of ESOS treatment entails complete surgical resection of the tumor with clear margins, accompanied by adjuvant chemotherapy. Ensuring precise diagnosis is of utmost importance to provide suitable and effective treatment

A Rare Autopsy Case of Aggressive Combined Hepatocellular-Cholangiocarcinoma with Stem Cell Features in A 59-Year-Old Female

Abstract Introduction/Objective Combined hepatocellular-cholangiocarcinoma (cHCC-CC) is a rare liver tumor with histologic and phenotypic features of both hepatocellular carcinoma and intrahepatic cholangiocarcinoma. It is believed to arise from hepatic progenitor cells. It consists of intermingled malignant biliary and hepatic tissue and thus is a distinct entity, rather than two separate coexisting malignancies. Methods/Case Report A 59-year-old female with a history of hepatitis C and cirrhosis presented with abdominal pain, shortness of breath, and altered mental status. She developed hematemesis and went into cardiac arrest. Massive transfusion and cardiopulmonary resuscitation were initiated. She was intubated, and an esophageal/gastric balloon was placed. She then underwent a procedure to relieve portal hypertension and related gastrointestinal bleeding. Despite extensive interventions, her condition continued to deteriorate. She expired one day after her initial presentation. Results (if a Case Study enter NA) At autopsy, the liver was diffusely and markedly fibrotic with numerous nodules of varying size with invasion into adjacent vasculature. Microscopic examination of the nodules revealed cHCC-CC with stem cell features and lymphovascular invasion. Further examination revealed tumor emboli scattered throughout the right lung. The patient’s history of chronic hepatitis C may have contributed to the development of her cHCC-CC. Although gross appearance resembled cirrhosis, upon microscopic inspection the nodules showed excessive tumor burden. Ultimately the patient had end stage liver disease due to the accumulation of damage and consequent fibrosis. This led to portal hypertension with subsequent massive gastrointestinal bleeding, hemorrhagic shock, and death. Conclusion cHCC-CC is a rare aggressive primary liver tumor with a poor prognosis. It can present with a cirrhotomemetic pattern with small nodules that can evade clinical and radiographic detection. Autopsy findings can provide valuable insights into the pathogenesis and clinical course of cHCC-CC, highlight the aggressive nature of the disease, and may inform future diagnostic and therapeutic strategies. Accurate diagnosis of this tumor is important for patient management and prognostication.

RADT-34. CLINICAL OUTCOMES OF LOCALISED AND METASTATIC CENTRAL NERVOUS SYSTEM HAEMANGIOPERICYTOMAS/SOLITARY FIBROUS TUMOURS – A SINGLE INSTITUTION EXPERIENCE

Abstract BACKGROUND/OBJECTIVE Central nervous system (CNS) haemangiopericytomas (HPC) or solitary fibrous tumours (SFT) are rare aggressive tumours. Surgery is the primary modality of treatment, however, often followed by postoperative radiotherapy due to high tendency for local recurrence. Although rare, extracranial metastases to several sites have been described. We aim to report our institutional experience in managing these rare tumours. METHODS Patients with central nervous system HPC/SFT who underwent surgical resection from 2012 to 2022 were included in the study. Data collected included patient demographics, tumour characteristics, treatment details (including primary surgery, radiotherapy and treatment for relapse) and clinical outcomes. RESULTS Five patients were included in this study. All were women and their mean age at diagnosis was 43 years. Four patients presented with intracranial disease and one with cervico-thoracic spine disease. All patients had undergone primary surgery and gross total resection was achieved in two patients. Two patients had grade 1, one with grade 2 and two with grade 3 HPC. Four patients received radiotherapy (median dose 60Gy) and one was on imaging surveillance after surgery. Three patients had relapsed – one with locoregional relapse only, one with distant (liver and bone) relapse and other with locoregional and distant (lung and bone). Time to relapse were 192, 39 and 120 months respectively. Stereotactic radiotherapy was used to treat local relapse in posterior fossa and dura of cervical spine, and metastatic relapse in lung with a stable radiological response. One patient with a painful destructive bone metastases was treated with palliative radiotherapy with good pain control. Four patients were alive at the time of the study, and one was lost to follow up. CONCLUSION We present our institutional experience in management of localised and metastatic HPC/SFT. Stereotactic radiotherapy could be considered an effective treatment modality for small volume relapse of HPC.

Smíšený adenoneuroendokrinní karcinom žaludku – kazuistika

Neuroendocrine tumours represent a heterogeneous group of neoplasia arising from different anatomical locations, with approximately 50% of gastrointestinal origin. Main parameters in the evaluation of each case include tumour morphology, mitotic cell count, and Ki-67 index. Mixed adeno-neuroendocrine carcinomas (MANECs) are rare aggressive neoplasms consisting of both adenocarcinomatous and neuroendocrine cells, each component constituting at least 30% of the lesion. Our case represents 77-year-old polymorbid patient who, due to signs of acute bleeding in the upper gastrointestinal tract with anaemic syndrome, underwent a gastroscopic examination for melena with the finding of an ulcer lesion on the front wall of the stomach at the junction of the body and the antrum. The control gastroscopic examinations with biopsies, at first only signs of chronic gastritis with Helicobacter pylori positivity were histologically proven, then fragments of high-grade tubular to tubulovillous adenoma and structures of moderately differentiated tubular adenocarcinoma were found. Histological analysis of the gastric resection showed mixed adeno-neuroendocrine carcinoma with lymphangioinvasion.

Primary Angiosarcoma of the Breast: A 20-Year Single-Institution Experience in China

Background. Primary angiosarcoma of the breast (PAS) is a rare aggressive tumor with no standardized treatment. The aim of this study was to investigate the characteristics of all primary angiosarcoma of the breast obtained from a single center and the features peculiar to Chinese patients. Methods. The medical records at Tianjin Medical University Cancer Institute and Hospital were retrospectively searched to identify all cases of PAS treated in 2000–2019. Results. Sixteen cases of PAS were identified, and most involved the left breast. Forty percent of young patients had a history of progressive tumor enlargement with localized pain and skin color changes. The diagnostic accuracy rate was 66.7% for MRI, 75% for core-needle aspiration, and 58.3% for intraoperative fast frozen pathology. The most common surgery was modified radical mastectomy (n = 9, 56.25%). All positive margins involved the pectoralis major muscle, and these tumors’ mean size was 8.2 cm. All cases were CD34 positive, and the Ki-67 index was ≥30% in 37.5%. Median local or distant recurrence-free survival was 57.6 months for low-to-moderate-grade tumors and 23.5 months for high-grade tumors. Seventy-five percent of the patients were treated with chemotherapy. The average tumor size in patients with relapse-free survival longer than 3 years was 2.2 cm. Conclusion. Young patients may have larger and softer breast tumors with skin color changes. MRI and core-needle biopsy should be performed preoperatively. A positive surgical margin at pectoralis major should be noted. Breast prosthesis may be a better reconstruction option. Adjuvant chemotherapy and/or radiotherapy should be considered for large tumors with a high Ki-67 index or high-grade tumors.

Reduced recurrence rate and comparable functionality after wide resection and reverse total shoulder arthroplasty with allograft-prosthetic composite versus curettage for proximal humeral giant cell tumor: a multicenter retrospective study

Giant cell tumors of bone (GCTBs) are rare, aggressive tumors, and the proximal humerus is a relatively rare location for GCTBs; limited evidence exists on which surgical approaches and reconstruction techniques are optimal. In the largest case series to date, we evaluated the recurrence rate of proximal humeral GCTBs and the functional outcomes of different resection and reconstruction options in this multicenter study. All 51 patients included in this study received initial surgical treatment for proximal humeral GCTBs from January 2007 to December 2020, with a minimum 2-year follow-up period. Local recurrence and functional outcomes were statistically analyzed in relation to demographic, clinical, and primary surgical variables. Functional outcomes were reported by patients and were assessed by the Musculoskeletal Tumor Society score and QuickDASH instrument (shortened version of the Disabilities of the Arm, Shoulder and Hand instrument). The mean follow-up period was 81.5 months (range, 30-191 months), and the overall recurrence rate was 17.6% (9 of 51 patients). The majority of recurrences (n=7) occurred in the first 2 years of follow-up. The intralesional curettage group (n=23) showed a statistically significant difference in the recurrence rate compared with the en bloc resection group (n=28) (34.8% vs. 3.6%, P=.007). Among shoulders receiving en bloc resection, 16 were reconstructed with hemiarthroplasty; 8, reverse total shoulder arthroplasty (rTSA) with allograft-prosthetic composite (APC) reconstruction; and 4, arthrodesis. On the basis of intention-to-treat analysis, the mean functional Musculoskeletal Tumor Society scores of the groups undergoing curettage, rTSA with APC, hemiarthroplasty, and arthrodesis were 26.0±3.1, 26.0±1.7, 20.3±2.8, and 22.5±1.3, respectively (P<.001 [with P<.001 for curettage vs. hemiarthroplasty and P=.004 for rTSA with APC vs. hemiarthroplasty]) and the mean QuickDASH scores were 14.0±11.0, 11.6±4.5, 33.1±11.8, and 21.6±4.7, respectively (P<.001 [with P<.001 for curettage vs. hemiarthroplasty and P=.003 for rTSA with APC vs. hemiarthroplasty]). On the basis of our data, en bloc resection followed by reverse shoulder arthroplasty showed a lower recurrence rate and no significant difference in functional outcome scores for proximal humeral GCTBs compared with intralesional curettage. Therefore, we believe that rTSA with APC may be reasonable for the initial treatment of proximal humeral GCTBs.

Surgical treatment of primary synovial lung sarcoma: a case report

Synovial sarcoma is a rare aggressive malignant mesenchymal tumor that accounts for 2.510.0% of all sarcomas and is most often found in the soft tissues of the extremities, especially near large joints. Primary synovial lung sarcoma is quite rare and accounts for 0.5% of all primary malignant lung neoplasms. The tumor is sensitive to chemotherapy, but the main method of treatment of synovial lung sarcoma remains surgical. Publications devoted to this topic, are presented by clinical observations due to the rare occurrence of the disease, therefore, each such observation is of scientific and practical interest.&#x0D; We present a clinical case 65-year-old patient with a tumor of the right lung with a diameter of more than 25 cm. Repeated attempts to perform transthoracic puncture both in our department and in other hospitals for the biopsy were unsuccessful. Taking into account the progression of the disease, the appearance and increase of the respiratory failure clinic, the impossibility to exclude the malignant nature of the process and the good functional status of the patient, a decision for surgery was made. The patient underwent right pneumonectomy with mediastinal lymph node dissection. The postoperative period was complicated by intrapleural bleeding, for which a rethoracotomy was performed. After recovery, the patient was discharged from the hospital in a stable condition.

Metastatic Medullary Thyroid Carcinoma without Evident Thyroid Nodules: A Rare Presentation

Introduction: Uncommon thyroid and neuroendocrine tumor; medullary thyroid carcinoma (MTC) is a rare, aggressive malignancy, with a 10-year survival rate of roughly 50%. Clinical presentation of MTC differs from sporadic to familial variety, but frequently it is showcased as a painless thyroid nodule. Metastatic medullar carcinoma without nodules is scanty, adding to the unpredictable behaviors of this entity. Case Presentation: Thus; the report of this rare presentation in 57-year-old patient with cervical metastatic lymphadenopathy, and a normal sized thyroid and no distant metastases, to which the patient underwent a chemo/ radiotherapy because the encompassment of the vascular axis. Conclusion: Metastasis medullary thyroid carcinoma with normal-size thyroid is somehow unheard of in the literature, as this rare aggressive tumor is usually diagnostic at a stage where the thyroid nodule is accompanied with lymph node metastases.