A Rare Case of Melanotic Schwannoma Occurred Intraosseous of Sacrum: A Literature Review.

BACKGROUND: Melanotic schwannoma is a rare tumor when it occurs in the sacrum. Though it is mostly classified as benign, the prognosis is unpredictable due to the possibility of recurrence and metastasis. Here, we reported a case of intraosseous of sacrum with good results and reviewed the literature. CASE PRESENTATION: A 33-year-old male patient complained of low back pain and was discovered to have an obstruction at S2. Following the necessary imaging diagnosis, we treated the patient with piecemeal excision in conjunction with extended curettage, and the frozen biopsy revealed that the tumor was melanotic schwannoma. The intraosseous portion of the lesion was curettaged using high-speed drill to enlarge the edge of curettage, and piecemeal excision for lesion within the sacral canal. After surgery, the patient received total 56Gy radiotherapy and frequent follow-up. After 15 months follow-up, there was no evidence of recurrence, and the nerve function was normal. CONCLUSION: Melanotic schwannoma that occurs intraosseous of the sacrum is extremely rare and lacks typical clinical manifestations; however it can be identified through careful pathological and imaging diagnosis. Intralesional extended curettage combined with radiotherapy can achieve a good local control with a satisfactory clinical effect in this rare disease.

Fresh Tissue Multi-omics Profiling Reveals Immune Classification and Suggests Immunotherapy Candidates for Conventional Chondrosarcoma.

PURPOSE: There is still no standard nonsurgical regimen for conventional chondrosarcoma (CHS). We aimed to identify whether any CHSs have a favored microenvironment for immunotherapy via multidimensional evaluation of the immunologic characteristics of this tumor. EXPERIMENTAL DESIGN: We obtained 98 newly-diagnosed CHS fresh tumors from several institutions and performed comprehensive analysis of data from CyTOF, whole-exome sequencing, and flow cytometry in 22 cases. Clinical data from immunotherapy responders and nonresponders were compared to explore possible biomarkers of immunotherapy response. Mechanism studies were conducted to interpret the biomarker phenotype. RESULTS: Based on the integrated data of single-cell CyTOF and flow cytometry, the CHS immune-microenvironment phenotypes were classified into three groups: subtype I, the "granulocytic-myeloid-derived suppressor cell (G-MDSC) dominant" cluster, with high number of HLA-DR- CD14- myeloid cells; subtype II, the "immune exhausted" cluster, with high exhausted T-cell and dendritic-cell infiltration; and subtype III, the "immune desert" cluster, with few immune cells. Immune cell-rich subtypes (subtype I and II) were characterized by IDH mutation, pathologic high grade, and peritumoral edema, while subtype I cases were exclusively featured by myxoid transformation. In clinical practice involving 12 individuals who received PD-1 antibody immunotherapy, all of the 3 cases with controlled diseases were retrospectively classified as subtype II. In mechanism, IDH mutation significantly elevated chemokine levels and immune-cell infiltration in immune-inactivated tumors. CONCLUSIONS: This study is the first to provide immune characterization of CHS, representing a major step to precise immunotherapy against this malignancy. Immunotherapy is promising for the "immune exhausted" subtype of patients with CHS.

Case Report: Radiological Features of Sclerosing Epithelioid Fibrosarcoma in the Right Fibula.

BACKGROUND: Sclerosing epithelioid fibrosarcoma (SEF) is an extremely rare, aggressive malignant subtype of fibrosarcoma. Only dozens of cases of primary SEF in the bone have been reported so far, without case involving fibula reported in literature to date. Herein we report the first case of primary SEF in the right fibula in a 19-year-old man. In this case report, we firstly give a comprehensive description of fibula SEF, including its complete clinical course and radiological findings. CASE PRESENTATION: A 19-year-old man presented with a half-year history of soreness in the right lower leg. Contrast-enhanced computed tomography (CE-CT) and magnetic resonance imaging (MRI) of the right lower leg were performed. Based on the radiological examinations, a diagnosis of malignant tumor arising in the fibular diaphysis was made. Final diagnosis of primary SEF in the right fibula was confirmed by histopathological and immunohistochemical examinations after surgical resection. The patient had no signs of recurrence or metastasis during a 24-month follow-up. CONCLUSION: We report an exceedingly rare case of primary SEF in the right fibula and its radiological features with CE-CT and MRI.

Clinical and laboratory characteristics of lymphoid neoplasms in serous effusions: a single centre experience in China.

BACKGROUND: Lymphoid neoplasms in serous effusions are uncommon, and the details of lymphoid neoplasms in serous effusions from China are still unclear. METHODS: Between January 2004 and December 2019, all patients with lymphoid neoplasms in pleural effusions, ascites, and pericardial effusions in our hospital, were reviewed. RESULTS: A total of 65 patients with lymphoid neoplasms were collected during this period. The top three neoplasms were diffuse large B-cell lymphoma (DLBCL) (n = 20, 30.7%), myeloma (n = 13, 20.0%), and T-lymphoblastic lymphoma (n = 7, 10.8%). In pleural effusions involving DLBCL, the cytomorphology of DLBCL cells was diverse; most pleural effusions were present during the tumor course (92.9%); bilateral pleural effusions were predominant (57.2%); and the median survival time was only 1.23 months after the effusion. In pleural effusions involving myeloma, 90.9% of cases (10/11) had a high ratio (> 1.0) of immature to mature plasma cells; paraprotein types of IgA (36.4%) and light chain λ (36.4%) were the most frequently found; bilateral pleural effusions were easily found (n = 10, 90.9%); and the median survival time was only 1.4 months after the effusion. CONCLUSION: In pleural effusions involving DLBCL, most of our patients with effusions are present during the tumor course, and bilateral pleural effusions are predominant. In pleural effusions involving myeloma, the paraprotein types of IgA and light chain λ are the most frequently found, and it has a high ratio of immature to mature plasma cells in pleural effusions.

Unusual findings of acute myeloid leukemia with inv(3)(q21q26.2) or t(3;3)(q21;q26.2): A multicenter study.

INTRODUCTION: AML with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2) [inv(3)/t(3;3)] was very rare. Currently, most reports of AML-inv(3)/t(3;3) were from Western countries, and few reports were from Asian countries. Racial differences in patients with AML-inv(3)/t(3;3) are still unknown. METHODS: Between January 1996 and April 2018, a total of 37 AML cases with inv(3)/t(3;3) were studied retrospectively. They were collected from 2229 primary AML cases performed with conventional cytogenetic analysis (37/2229, 1.66%). RESULTS: Here, some differences were found by comparing our data with those from Western countries. In our series, AML with inv(3)(q21q26) had a lower incidence than that with t(3;3)(q21;q26) (11 vs 26 cases). Our patients seemed to be more younger (median, 43 years) and have lower hemoglobin concentrations (median, 73 g/L) and higher platelet count (median, 351 × 109 /L). A higher incidence of acute monoblastic and monocytic leukemia (45.9%) was observed in our patients. Immunophenotypic studies showed that CD38 (30.8%) was not so frequently expressed as that in the earlier reports. Mutations analysis showed a high frequency of NRAS mutations (45.0%), followed by SF3B1(15.0%), GATA2(15.0%), FLT3-ITD(10.0%), C-Kit/D816(5.0%), and CEBPA(5.0%), without mutation of NPM1(Exon12)or JAK2V617. CONCLUSION: Ethnic differences do exist between the Chinese and Western patients with AML-inv(3)/t(3;3), and more attention should be paid involving different ethnic populations and geographic regions.

Lymphadenitis associated with cat-scratch disease simulating a neoplasm: Imaging findings with histopathological associations.

The lymphadenitis associated with cat-scratch disease (CSD) is often confused with neoplasms by a number of radiologists and clinicians, and consequently, unnecessary invasive procedures or surgeries are performed. In the present study, the contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) findings of 10 patients (6 men and 4 women) with clinically and pathologically confirmed lymphadenitis associated with CSD were retrospectively analyzed (CT in 3 patients, MRI in 6 patients, and CT and MRI in 1 patient) at The Second Affiliated Hospital of Zhejiang University School of Medicine (Hangzhou, China) between January 2007 and November 2014. As a result, 17 enlarged lymph nodes were identified in 10 cases. The 5 nodes identified by CT scan exhibited relatively inhomogeneous isodensity to muscle, with patchy low density in the center. All 14 nodes identified by MRI scan exhibited homogeneous or heterogeneous isointensity to muscle or slightly increased intensity compared with that of muscle on T1-weighted images (T1WI), and homogeneous or heterogeneous hyperintensity on fat-suppressed T2WI. Following enhancement, all 17 enlarged lymph nodes associated with CSD demonstrated the following 3 different enhancement patterns: Moderate homogeneous enhancement (n=8), which was associated with histologically identified early disease stage; marked heterogeneous enhancement with no enhancement of the necrotic areas (n=4), and heterogeneous enhancement with progressively 'spoke-wheel-like' (defined as radiating enhancement from the center) enhancement of the patchy low-density area (n=1), which was associated with histologically identified intermediate disease stage; and astral low-density/hypointensity with marked enhancement (n=2) or a 'rose flower' sign (n=2), which was associated with histologically identified late disease stage. We hypothesized that the CT and MRI results of lymphadenitis in CSD may be associated with the pathological features. It may be suggested that the diagnosis of CSD may be formed when considering the characteristic CT and MRI features of astral low-density/hypointensity with marked enhancement or a 'rose flower' sign (defined as marginal petaloid enhancement) in the late disease stage, or the MRI results of homogeneous, moderate enhancement in the early disease stage, or the CT/MRI data of heterogeneous enhancement with non-enhancing area in the center in the intermediate disease stage, in solitary or multiple enlarged lymph nodes associated with general subcutaneous edema in the vicinity of the nodes on CT/MRI and with a history of cat exposure.

Contrast-enhanced multiple-phase imaging features of intrahepatic mass-forming cholangiocarcinoma and hepatocellular carcinoma with cirrhosis: A comparative study.

The intrahepatic mass-forming cholangiocarcinoma (IMCC) is frequently misdiagnosed as hepatocellular carcinoma (HCC) in patients with cirrhosis, by numerous radiologists and clinical doctors, which results in the incorrect therapeutic treatment. A retrospective case-control study was conducted, and the contrast-enhanced multiple-phase (CEMP) computed tomography (CT) and magnetic resonance imaging (MRI) findings of 22 pathologically confirmed IMCC patients and 22 HCC controls with underlying liver cirrhosis were analyzed at the present hospital, from January 2010 to December 2015. In addition, serum tests were conducted and clinical symptoms of patients evaluated. A statistical analysis revealed that the enhancement pattern, signal on MRI delayed phase (P<0.001), maximum diameter, capsule retraction, portal vein invasion, bile duct dilation and abdominal lymphadenectasis characteristics were different between IMCC and HCC patients with cirrhosis. On CEMP CT and MRI analysis, the most frequently occurring enhancement patterns of IMCC were progressive patterns (P=0.001 or P<0.001). Conversely, the most frequently occurring enhancement patterns present in HCC were the washout patterns (P<0.001). Therefore, the diagnosis of IMCC in cirrhotic patients should be verified with CEMP CT and MRI analysis for the future, to determine presence or absence of progressive and/or peripheral rim-like enhancement, a hyperintensive delayed phase with capsule retraction, portal vein invasion, bile duct dilation, abdominal lymphadenectasis and increased levels of CA199.

Differential CT and MR imaging diagnosis between low- and high-grade malignant vascular tumors of bone.

OBJECTIVE: To investigate the CT and MR imaging findings and differential diagnosis of malignant vascular tumors of bone. MATERIALS AND METHODS: CT and MR imaging findings of 18 patients with histopathology-proven malignant vascular tumors of bone were examined. Assessed image features included age, sex, location, CT findings, and MR imaging appearances and dynamic contrast-enhanced MR imaging. RESULTS: The study group comprised 18 cases, of which 7 were categorized as low-grade malignant vascular tumors (LMT), and 11 were categorized as high-grade malignant vascular tumors (HMT). Malignant vascular tumors of bone showed osteolytic lesions with heterogeneous signs and enhancement, and frequently associated with soft tissue masses and peritumorous edema. The mean age of patient was respectively 34.7 years in LMT with 3 patients younger than 20 and 49.9 years in HMT with 3 patients older than 60 years. The mean lesion diameter was 3.6 cm (range 2-7.2) in LMT with two lesions <3 cm and 7.1 cm (range 3-13) in HMT with 3 lesions greater than 10 cm. LMT showed multifocal (57.1 %) and well-defined (71.5 %) lesions with residual bone (57.1 %), peripheral sclerosis (85.7 %), and slightly hetergeneous enhancement (71.4 %), compared to those of HMT with 9.1, 45.5, 27.3, and 72.7 %, and 9.1 % respectively. Also, HMT appeared as expansive (54.5 %), ill-defined (54.5 %), macroscopic necrosis/cystic (81.8 %) or hemorrhagic (27.3 %) lesion with pathological fracture (27.3 %), and often presented with obviously hetergeneous enhancement (81.8 %), compared to those of LMT with 42.9, 28.6, 42.9, 0, 14.3 and 14.3 % respectively. CONCLUSIONS: There are some differences in the imaging features between LMT and HMT, while unifocal/multifocal, expansive, ill-defined, necrosis/cystic, hemorrhagic features with age, lesion diameter, peripheral sclerosis, residual bone tissue, pathological fracture and slightly/obviously hetergeneous enhancement highly suggest their differential diagnosis.

Epithelioid hemangioma of bone: a report of two special cases and a literature review.

Intraosseous epithelioid hemangioma (EH) is a rare intermediate vascular neoplasm, characterized by locally aggressive and rarely metastasizing behavior. Occasionally, EH of bone can behave strangely and may simulate malignant neoplasm. Here, we report two cases of EH of bone. Of interest was the fact that the computed tomography and magnetic resonance images from one case showed an osteolytic lesion in the right scapula, with multiple swollen lymph nodes in the right supraclavicular and axillary areas. Another patient exhibited a local recurrence in the cervical vertebrae. The initial radiological diagnosis of both cases was metastatic tumor. EH should be included in the differential diagnosis of a radiographic osteolytic lesion with an aggressive appearance. Also, we reviewed the literature that reported EH of bone and summarized their radiological appearances. The cases of EH of bone that exhibited involvement of regional or draining lymph nodes were also summarized.

Breast malignant phyllodes tumor with rare pelvic metastases and long-term overall survival: A case report and literature review.

BACKGROUND: Malignant phyllodes tumor (PT) is a rare fibro epithelial neoplasm of the breast, which is poor prognosis due to high risk of recurrence and distant metastasis. METHODS: We report a case of malignant PT. It had recurred locally five times, and the sixth relapse was occurred 54 months after first diagnosis, presenting a huge pelvic mass (14 cm × 11 cm) by CT scan. Histopathological examination has demonstrated a metastatic phyllodes tumor. After postoperative chemotherapy treatment, a longer survival has been achieved, which is more than 72 months. RESULTS: Our case report describes a breast PT with several local recurrences and a rare metastasis (pelvic cavity), but long-term overall survival was achieved after surgery and chemotherapy. CONCLUSION: We conclude that trustworthy prognosticators that identify patients with excessive potential of aggressive clinical course should be explored. Moreover, proper treatment could prolong overall survival of metastatic PT patients.

Multiple-phase spiral CT findings of pancreatic vasoactive intestinal peptide-secreting tumor: A case report.

The present study reports a case of pancreatic vasoactive intestinal peptide-secreting tumor (VIPoma), of 2.2 cm in diameter, arising from the region of the uncus of the pancreas with liver metastases in a 50-year-old patient, which demonstrated unusual multiple-phase spiral computed tomography (MPSCT) results. The pancreatic lesion was isodense compared with the pancreatic parenchyma. During the hepatic artery phase and portal venous phase, the mass was hypodense compared with the enhanced pancreas, with the mean CT attenuation (HU) values being 56 HU and 66 HU, respectively. During the hepatic parenchymal phase, the mass became hyperdense with the mean CT attenuation values being 74 HU. The process of contrast-enhanced MPSCT demonstrated progressive strengthening. Pancreaticoduodenectomy and wedge resection of a number of the liver metastases was performed. There are only a limited number of studies reporting CT findings of pancreatic VIPoma and no MPSCT findings have been described previously.

Bone multicentric epithelioid hemangioendothelioma of the lower and upper extremities with pulmonary metastases: A case report.

The present study reports a rare case of bone multicentric epithelioid hemangioendothelioma (EH) involving the upper and lower extremities simultaneously, with visceral involvement of the lung. Osteolytic lesions were first identified in the right distal femur and proximal tibia. Slight increased radionuclide uptake was observed in the right shoulder joint on bone scintigraphy, however, this was ignored, as no clinical symptoms were present. The patient was initially misdiagnosed with multifocal chondroblastoma, and an extra-articular curettage of lesions was performed in the proximal tibia and medial femoral condyle, which was filled with bone cement. The histopathological diagnosis was corrected post-operatively following immunohistochemical analysis, which indicated EH, and subsequently, an amputation of the right leg at thigh level was performed. In addition, multiple lytic lesions in the right shoulder joint and pulmonary metastases were identified on whole-body radiological examination. Radiotherapy was administered to the right shoulder joint, however, the patient refused chemotherapy or further surgery. At 15 months after the initial surgery, the patient currently remains alive. This case indicates that an improved understanding with regard to the clinical features of this disease may prevent misdiagnosis and improve EH treatment.

Clinicopathologic characteristics of prefibrotic-early primary myelofibrosis in Chinese patients.

The clinicopathologic features of patients with prefibrotic-early primary myelofibrosis (PEPMF) are still uncertain, and the characteristics of PEPMF in Asian patients are rarely reported. This study analyzed the clinicopathologic characteristics of 42 Chinese patients with PMF newly diagnosed according to the 2008 World Health Organization criteria. Some clinical and laboratory features of the patients differed significantly from those of the predominantly white patients in Western countries. Chinese patients with PEPMF were more often male (1.28:1) and younger, less likely to have higher median hemoglobin concentrations (126 g/L), less frequently had palpable spleens (35.7%), and had longer median times between prefibrotic-early and classical PEPMF (64 months). On bone marrow trephine sections, Chinese patients were more likely to have increased granulopoiesis (78.6%) and less frequently had balloon-like megakaryocytes (61.9%), giant and staghorn megakaryocytes (35.7%), or megakaryocytes with hyperchromatic and dysplastic nuclei (40.4%). In conclusion, some clinicopathologic characteristics of PEPMF in Chinese patients in China differ substantially from those seen in predominantly white patients in Western countries, and more clinicopathologic studies involving different ethnic populations and geographic regions of the world should help unfold the characteristics of this disease.

Hemolymphangioma: a rare differential diagnosis of cystic-solid or cystic tumors of the pancreas.

We report a case of pancreatic hemolymphangioma. Hemolymphangioma is a malformation of both lymphatic vessels and blood vessels. The incidence of this disease in the pancreas is extremely rare. To the best of our knowledge, only seven cases have been reported worldwide (PubMed). A 39-year-old woman with a one-day history of abdominal pain was admitted to our hospital. There was no obvious precipitating factor. The preoperative examination, including ultrasonography and computed tomography, showed a cystic-solid tumor in the pancreas, and it was considered to be a mucinous cystadenoma or cystadenocarcinoma. Pancreatic body-tail resection combined with splenectomy was performed. After the operation, the tumor was pathologically demonstrated to be a pancreatic hemolymphangioma. Although pancreatic hemolymphangioma is rare, we believe that it should be considered in the differential diagnosis of cystic-solid tumors of the pancreas, particularly when there is no sufficient evidence for diagnosing cystadenoma, cystadenocarcinoma or some other relatively common disease of the pancreas.