Papillary renal neoplasm with reverse polarity: an observational study of histology, immunophenotypes, and molecular variation.

Background: Papillary renal neoplasm with reverse polarity (PRNRP) is a novel entity with unique clinicopathological characteristics, and only a small number of patients with PRNRP have been described. Methods: We retrospectively analyzed the data for nine patients with PRNRP and evaluated differences in the clinical, histomorphological, immunohistochemical, and molecular features; prognosis; and differential diagnosis of PRNRP from other renal tumors with papillary structure. Results: There were six males and three females aged 36 to 74 years (mean: 62.33 years; median: 68 years). All the tumors were solitary and ranged from 1 to 3.7 cm (mean: 2.17 cm; median: 2 cm), with three and six tumors arose in the left and right renal tract, respectively. Pathologically, PRNRP is a small, well-circumscribed neoplasm with predominant papillary formations. The lining epithelium is composed of a monolayer of cuboidal to low-columnar cells with low-grade nuclei arranged against the apical pole of the tumor cells. Edema, mucinous degeneration, and hyaline degeneration are found in the fibrovascular cores. Foamy macrophages, psammoma bodies, hemosiderin deposition, and infiltrative tumor boundaries were present in some patients. Immunohistochemically, all tumors showed diffuse positive staining for GATA3. Sanger sequencing confirmed the presence of KRAS mutation in seven patients. All patients had a good prognosis after surgery and were relapse free. Positive staining for GATA3 and negative staining for vimentin were the most significant markers for differentiating PRNRP from other renal tumors with analogous structure. Conclusions: These findings suggested that PRNRP is a distinctive subtype of renal tumor with specific pathological features and indolent behaviors that should be distinguished from other renal tumors, especially papillary renal cell carcinoma. A monolayer of tumor cells with an inverted nuclear pattern, positive staining for GATA3, and KRAS mutation are essential for pathological diagnosis. Owing to its satisfactory prognosis, the surveillance and follow-up of patients with PRNRP should be additionally formulated.

Outcomes of endoscopic submucosal dissection versus esophagectomy for poorly differentiated superficial esophageal squamous cell carcinoma: A 10-year cohort study.

BACKGROUNDS: The efficacy of endoscopic submucosal dissection (ESD) to treat poorly differentiated superficial esophageal squamous cell carcinoma (SESCC) is unclear. AIMS: To exploring the efficacy and prognosis of ESD treatment poorly differentiated SESCC compared with esophagectomy. METHODS: A retrospective cohort study was conducted, the data of poorly differentiated SESCC patients who received ESD or esophagectomy from Jan 2011 to Jan 2021 were analyzed. Overall survival (OS), disease-specific survival (DSS), recurrence-free survival (RFS), and procedure-related variables were compared between ESD and esophagectomy group. RESULTS: 95 patients underwent ESD, while 86 underwent esophagectomy. No significant differences were found between the two groups in OS (P = 0.587), DSS (P = 0.172), and RFS (P = 0.111). Oncologic outcomes were also similar between the two groups in propensity score-matched analysis. For T1a ESCC, the rates of R0 resection, LVI or nodal metastasis and additional therapy were similar between ESD and esophagectomy groups. But for T1b ESCC, the rates of positive resection margin and additional therapy were significantly higher in ESD group than those in esophagectomy group. CONCLUSIONS: ESD is a minimally invasive procedure that has comparable oncologic outcomes with esophagectomy for treatment poorly differentiated T1a ESCC. However, ESD is not suitable for poorly differentiated T1b ESCC, additional surgery or radiochemotherapy should be required.

Rhabdoid glioblastoma: case report and literature review.

Rhabdoid glioblastoma is a recently described entity in which a glioblastoma is associated with a rhabdoid component. Although rhabdoid glioblastoma has not appeared in the new World Health Organization classification of tumors of the CNS, it has a specific morphological feature and highly aggressive clinic process. Up to now, there have been six cases of rhabdoid glioblastoma reported in the literature. We report rhabdoid glioblastoma in the right front temporal lobe from a 31-year-old Chinese man. This tumor consisted of rhabdoid tumor cells with an eccentric nucleus and an eosinophilic cytoplasm. The tumor had an area appearing to be glioblastoma with microvascular proliferation and necrosis, and lacked a primitive neuroectodermal tumor component, and a mesenchymal component. Vimentin, epithelial membrane antigen, GFAP and integrase interactor (INI-1) expression were found in the tumor cells. Genetic abnormalities which include monosomy or a deletion of chromosome 22 were not found in this tumor. After 3 months post-surgery, the tumor was widespread in leptomeningia and the patient died. In conclusion, rhabdoid glioblastoma is a rare glioblastoma with poor prognosis; the differential diagnosis contained other rhabdoid tumors. This case will contribute to the profile of rhabdoid glioblastoma with typical morphology and immunophenotype, genetic and clinic features.

Rupture of splenic artery aneurysm in pregnancy: a review of the literature and report of two cases.

BACKGROUND: Splenic artery aneurysms are an uncommon form of vascular disease, which have a significant potential for rupture, most commonly associated with pregnancy, typically presents as sudden, unexpected death. As a consequence, the initial recognition and diagnosis of splenic artery aneurysm rupture take place only at autopsy. CLINICAL CASES: This report presents 2 cases of sudden death resulting from splenic artery aneurysm in a pregnant woman and a postpartum woman, respectively. The former splenic artery aneurysm were measuring 1 cm in diameter and the latter splenic artery aneurysm 5.5 x 5 x 2 cm in size. Histologic examination of the both vessels wall showed severe morphologic changes of degeneration together with an attenuation of arterial internal elastica. CONCLUSIONS: To our knowledge, splenic artery aneurysm in pregnant woman is unusual vital disease. It is essential that obstetricians are alert to the prodromal and catastrophic symptoms of splenic artery aneurysm. Early recognition and prompt management, including early resected electively, are vital to the survival of both mother and fetus.

Primary cutaneous marginal zone B-cell lymphoma with amyloid deposition: report of two cases with review of literature.

BACKGROUND AND OBJECTIVE: Amyloid deposition is rare. If there was a great amount of amyloid depositions in the skin tissue, it would be considered to be amyloid deposition disease at first, and then primary cutaneous marginal zone B-cell lymphoma (PCMZL). This study was to analyze the diagnosis and differential diagnosis of two cases of PCMZL with amyloid deposition. METHODS: Clinicopathologic characteristics and follow-up of two cases of PCMZL were analyzed. Immunohistochemical staining was performed by EnVision method using antibodies LCA, CD19, CD20, CD79a, CD3, CD7, MUM1, kappa, lambda, Ki-67. IgH and TCRgamma gene rearrangement was detected by polymerase chain reactive (PCR). RESULTS: Case 1, a 71-year-old Chinese male, had a subcutaneous mass on the right elbow that was initially diagnosed with "amyloidosis" in 2004. Three years after the initial diagnosis, he developed recurrences on the right para-auxillary that was still diagnosed with "probably amyloidosis". Four years after the first diagnosis, the patient presented a lesion on the right para-auxillary with a diameter of 2 cm and a lesion on the temporal-parietal dural with a size of 6.0 cmx3.0 cmx3.0 cm. Case 2, a 68-year-old Chinese male, had a subcutaneous mass next to back of the left ear with a size of 9.0 cmx5.0 cm, and he underwent a operation one year previously because of subcutaneous mass in the same site. Microscopically, the tumors of both cases were located in dermis and subcutaneous, tumor cells were medium size with a nodular or diffuse distribution, and some of tumor cells were plasmacytoid/plasma cells. Morphologically, the temporal-parietal dural lesion was similar to subcutaneous lesion and infiltrated into cranial (case 1). Juxtaposed the tumor cells of two cases, there were the large amyloid deposits of amorphous hyaline material and concentrically laminated hyaline spherules in case 1, while cord-like amyloid deposits in case 2. Reactive lymphoid follicles with germinal centers and foreign body giant cells in the stroma were found surrounding the amyloid deposits. Congo red staining showed positive of amyloid deposition in tumor tissues of both cases. Immunohistochemical staining revealed that LCA, CD19, CD20, CD79a and MUM1 expressions were positive in tumor cells, and Ki-67 expression was about 8%-10%. IgL restricted expression as kappa positive while lambda negative was found in both cases. PCR results showed monoclone gene rearrangement of IgH gene in both cases. CONCLUSIONS: Our findings suggest that amyloid deposition rarely present in both primary and metastatic tumors in PCMZL, and its diagnosis should be considered to avoid misdiagnosis. The patients with PCMZL should undergo regular examinations and chemotherapy as well as a long-term follow-up since it is apt to recur or relapse.

[Solitary plasmacytoma of spine: a clinical, radiologic and pathologic study of 13 cases].

OBJECTIVE: To study the clinical, radiologic and pathologic features of solitary plasmacytoma of spine. METHODS: The clinical, radiologic and pathologic features, as well as treatment and follow-up data, of 13 solitary plasmacytoma of spine cases were retrieved and analyzed. Immunohistochemical study using EnVision method for LCA, CD19, CD20, CD79a, CD3, CD7, PC, MUM1, CD138, IgG, IgM, kappa, lambda and Ki-67 was carried out. RESULTS: All the tumours were primarily located in the vertebrae (including 9 cases in thoracic vertebrae and 4 cases in lumbar vertebrae). The male-to-female ratio was 3.3:1. The age of the patients ranged from 42 to 69 years (mean age = 56 years). The commonest symptom was pain in the surrounding regions. The degree of neurologic disturbance mostly depended on the extent of vertebral destruction and structural instability of the spine. Radiologic examination showed mainly osteolytic lesions in vertebrae. Magnetic resonance imaging demonstrated the presence of heterogeneous intensity inside the involved vertebrae (low in T1 weighted and high in T2 weighted images). Histologic examination showed diffuse infiltration by malignant cells. In well-differentiated plasmacytomas, the tumor cells resembled normal plasma cells. In poorly differentiated examples, the cellular morphology mimicked that of the centroblasts. The interstitial stroma was scanty and contained plenty of vessels, sometimes with formation of blood lakes. Amyloid deposition was present in some of the cases. Immunohistochemical study showed that the tumor cells were positive for CD79a and negative for CD20. Light chain restriction was detected in all the 13 cases studied. Plasma cell marker PC was expressed in all cases, while IgG was positive in 5 cases, IgM in 1 case, MUM1 in 10 cases and CD138 in 8 cases. Ki-67 index varied from 10% to 50%. All cases were operated, with adjuvant chemotherapy and radiotherapy given. CONCLUSIONS: Correlation of clinical, radiologic and pathologic features is important in diagnosis of solitary plasmacytoma of spine. The possibility of multiple myeloma needs to be excluded. Early detection by radiologic examination, local surgical resection, post-operative chemoradiotherapy and long-term follow-up are prudent for successful management of this condition.

Primary lymphoblastic B-cell lymphoma of the stomach: a case report.

Primary stomach lymphoblastic B-cell lymphoma (B-LBL) is a rare tumor. We describe a primary stomach B-LBL in a 38 years old female who presented with nonspecific complaints of fatigue and vomiting for 2 mo. Gastrofiberscopy revealed a large gastric ulcer, which was successfully resected. Pathology showed a lymphoblastic cell lymphoma arising from the stomach, and there was no evidence of disease at any extrastomach site. Immunohistochemical staining and gene rearrangement studies supported that the stomach tumor was a clonal B-cell lymphoma. Therefore, the diagnosis of B-LBL was made based on the stomach specimen.

Gastric involvement of extranodal NK/T-cell lymphoma, nasal type: a report of 3 cases with literature review.

Gastric involvement of extranodal natural killer/T-cell lymphoma, nasal type is extremely rare, and its clinicopathologic features are also poorly understood. Until now, only 4 cases have been reported in literature. In this article, 3 cases of extranodal natural killer/T-cell lymphoma, nasal type are reported. In this current series, one patient (case 2) was admitted to emergency due to life-threatened hemorrhage of the upper-digestive tract and the volume of blood loss was about 2000 mL. For case 3, the sample was a small piece of gastroendoscopy biopsy tissue. It is a challenge for pathologist to make the diagnosis for this special type of tumor. In all, 2 of 3 cases had a relevant history of midfacial extranodal natural killer/T-cell lymphoma, nasal type before. The other patient died of tumor 2 months later with no history of the tumor when she was alive. Literature review was carried out; The cases reported in this article are documented and compared with the 4 previously reported cases of tumor.

Secondary skin involvement in gastric diffuse large B-cell lymphoma treated with chidamide: A case report.

RATIONALE: Diffuse large B-cell lymphoma (DLBCL) is a neoplasm of large B lymphoid cells that exhibits diffuse growth patterns. Patients may present with nodal and/or extranodal disease. The most common extranodal site is the gastrointestinal tract, while skin is less common. PATIENT CONCERNS: We report a case of secondary skin involvement of an original gastric DLBCL, which has achieved a complete response after treatment with chidamide. DIAGNOSES: Initially, the diagnosis of gastric DLBCL is clear, and this patient responded well to systemic chemotherapy (rituximab + cyclophosphamide + epirubicin + vincristine + prednisone) after 8 cycles. Thirty months later, some rapidly enlarging skin nodules on his arm were found. These skin nodules were diagnosed as secondary cutaneous DLBCL based on the clinical features, positron emission tomography-computed tomography, and histomorphologic and immunohistochemical expression. INTERVENTIONS: Steroids, interferon-α, and radiation had little therapeutic effect. We treated the patient with chidamide at 30 mg twice per week in combination with dexamethasone. OUTCOMES: The skin nodules regressed 3 weeks later. During the 1-year follow-up period, the patient is still in treatment with chidamide without adverse reactions. LESSONS: To the best of our knowledge, this is the first case of secondary skin DLBCL reported to exhibit a complete response to chidamide, which provides a novel therapeutic strategy for secondary skin DLBCL. However, more cases are still needed to further validate its efficacy.

Establishment of a novel human lymphoblastic cell strain with the long arm of chromosome 11 aberration without MLL rearrangement.

At present, all cell strains derived from acute lymphoblastic leukemia (ALL) patients with the long arm of chromosome 11 aberration are accompanied with mixed lineage leukemia (MLL) gene rearrangement. In this study, we established a permanent ALL cell strain CHH-1 with the long arm of chromosome 11 aberration and without MLL rearrangement, hoping that it could be used for the research of ALL with such genetic abnormality. CHH-1 cell strain was certified through morphology, immunophenotype, genetics and immunoglobulin (Ig) gene rearrangement analysis. Cell characteristics including tumorigenic ability, semisolid colony forming ability, telomerase activity, autocrine and invasion were further detected. Cells were with an add(11)(q23) structural abnormality without MLL rearrangement, and were consistent with the genetic abnormality of the patient. In addition, these cells had features of tumor-forming ability, high colony forming capacity, unique cytokine autocrine mode, high telomerase activity, and high invasion ability. CHH-1 may prove to be a useful cell model for the research of human leukemia with genetic aberration in chromosome 11, and help explore the role of such genetic abnormality in the pathogenesis, progression and prognosis of ALL, and in developing new target drugs.

[Real-time fluorescence quantitative PCR in detecting ribosome protein S13 (RPS13) gene expression in NK/T cell lymphoma].

OBJECTIVE: To quantitatively detect the expression level of ribosome protein S13 (RPS13) in NK/T cell lymphoma. METHODS: The total RNA isolated from the parafin-embedded tissue of NK/T cell lymphoma was reversely transcribed into cDNA. The real-time fluorescence quantitative PCR technology method was used to analyze the expression level of RPS13 gene. RESULTS: The real-time fluorescence quantitative PCR technology was successfully performed to precisely detect the mRNA level. The expression level of RPS13 gene in tumor tissue of 20 cases of NK/T cell lymphoma was dramatically lower than that in normal peripheral blood lymphocyte of healthy controls. CONCLUSION: The RPS13 gene has lower expression level in tumor tissue cells of NK/T cell lymphoma than in normal lymphocyte, indicating that it plays an role in the development of the NK/T cell lymphoma.

Peripheral T-cell lymphoma at the injection site of influenza vaccination.

Pseudolymphomas or B-cell lymphoma at the vaccination site have been reported by several authors. However, onset of cutaneous T-cell lymphoma with cytotoxic features is a rare complication of vaccination. We report a 27-year-old man who developed a nodule and ulcer that arose at the site of injection of influenza vaccine. The neoplastic cells reacted positively for CD56, CD3, CD2, perforin, and granzyme B, but negatively for CD4, CD8, CD10, CD19, CD30, CD34, CD79, and betaF1. Molecular studies showed T-cell receptor γ (TCR-γ) chain monoclonal rearrangement. A diagnosis of peripheral T-cell lymphoma, not otherwise specified (NOS) was established. The patient had high fever, progressive liver dysfunction and a rapid fatal evolution.