Anomalous unilateral single pulmonary vein: Two cases mimicking arteriovenous malformations and a review of the literature

Total anomalous pulmonary venous drainage is a rare congenital anomaly. It usually involves a pulmonary to systemic venous shunt and most cases have a septal defect in order to survive. Anomalous pulmonary venous drainage with pulmonary venous shunting is an extremely rare and entirely benign entity. We present two such cases, in which there was atresia of the left superior pulmonary vein and drainage via a tortuous collateral vein to the left inferior pulmonary vein. This collateral was mistaken on plain film and CT for a pulmonary arteriovenous malformation. Awareness of this anomalous unilateral single pulmonary vein and its radiological appearances may help in avoiding unnecessary pulmonary angiography.     

Robinow syndrome: Report of two cases and review of the literature

We report two patients with Robinow syndrome, review the published literature and stress the importance and limitations of radiographic examination in the diagnosis of this disorder, which shows extreme clinical and radiographic variability. The radiographic differential diagnosis of Robinow syndrome is discussed.     

Spontaneous tumor lysis syndrome in acute myeloid leukemia: two cases and a review of the literature

Spontaneous tumor lysis syndrome (TLS) is a constellation of electrolyte abnormalities and acute renal failure, which occurs in the setting of rapid cell turnover prior to the administration of cytotoxic chemotherapy. While spontaneous TLS is well described in patients with Burkitt's lymphoma, it is thought to occur less commonly in other hematologic malignancies. We present two cases of spontaneous TLS in patients with newly diagnosed acute myeloid leukemia (AML) followed by a review of the literature in this field.     

Portal vein thrombosis during antineoplastic chemotherapy in children: report of five cases and review of the literature

We report five paediatric cases of portal vein thrombosis (PVT) occurring during chemotherapy, observed in two institutions over an 8-year time period. These children aged 2.5–15 years were treated for Burkitt’s lymphoma, Ewing’s tumour, small cell bone tumour or medulloblastoma. PVT was diagnosed on colour Doppler ultrasonography (US). In four patients, thrombosis occurred 2–45 days after severe hepatic veno-occlusive disease (HVOD) secondary to intensive chemotherapy containing busulfan. In one case, PVT occurred in the absence of HVOD in a patient with pre-existing periportal lymphomatous infiltration. Four patients experienced persistent portal hypertension, which resulted in death in one. PVT during chemotherapy in children is a rare event and appears to be closely related to intensive chemotherapy containing busulfan and to be associated with HVOD.     

Classic biphasic pulmonary blastoma: a case report and review of the literature

Pulmonary blastomas are rare malignant tumors, comprising only 0.25-0.5% of all malignant lung neoplasms. Pulmonary blastomas are subdivided in three categories: well-differentiated fetal adenocarcinoma (WDFA), classic biphasic pulmonary blastoma (CBPB) and pleuropulmonary blastoma (PPB), which is currently regarded as a separate entity. The majority of patients with CBPB and WDFA are adults with an average age of 43 years. Tobacco use is identified as a causative agent. Symptomatology varies from asymptomatic (40%) to symptoms of a non-specific pulmonary disease. The most common roentgenologic pattern is a large peripheral nodule. The treatment of choice is surgical excision. The efficacy of adjuvant chemotherapy and radiotherapy is not yet established. The prognosis of pulmonary blastoma is very poor; overall five-year survival is 16%. WDFA appears to have a better prognosis. Adverse prognostic factors are biphasic type, tumor recurrence, metastasis at initial presentation, gross size of the tumor (>5 cm) and lymph node metastasis. On the basis of the available literature, an initial aggressive treatment that includes surgery and, wherever possible, postoperative chemotherapy and radiotherapy could be useful to prolong survival in patients with this rare lung neoplasm. We present a case of classic biphasic pulmonary blastoma in a 77-year old male and review the literature.     

Comparison in treatments of nonleukemic granulocytic sarcoma: report of two cases and a review of 72 cases in the literature

BACKGROUND: The purpose of this study was to reveal the clinical characteristics of nonleukemic granulocytic sarcoma (GS) and an association between the therapeutic regimens and the nonleukemic period. METHOD: Clinical records of 2 patients reported here and 72 patients gathered using a literature search on Medline from other institutions were analyzed. The patients consisted of 57 patients who preceded acute nonlymphoblastic leukemia (ANLL) and 17 patients who did not develop ANLL. These patients were divided into 3 groups by therapeutic regimens; Group I included 12 patients who received only biopsy or surgical resection of the tumor, Group II was 20 patients who received local irradiation for the tumor, and Group III consisted of 42 patients who received systemic chemotherapy. The nonleukemic periods between these groups were compared. In Group III, the period in the patients who were treated with chemotherapy given to ANLL was compared with that in the patients who received chemotherapy used for malignant lymphoproliferative disorders (MLPDs). RESULTS: Thirty-five patients (47%) initially were misdiagnosed, and the disease was most often malignant lymphoma. Preferential sites of GS were the small intestine, mediastinum, epidural site, uterus, and ovary, which often are difficult for the detection and diagnosis in addition to the skin and lymph nodes known commonly. The nonleukemic period after the diagnosis of GS was significantly longer in Group III than in the other groups (median, 12 months in Group III vs. 3 and 6 months in Groups I and II, respectively). The aggressive chemotherapy given to ANLL led to a longer nonleukemic period than the chemotherapy used for MLPDs. CONCLUSIONS: To reduce the risk of subsequent ANLL in patients with nonleukemic GS, it is important that accurate histologic diagnosis is established initially for GS and that all isolated cases of GS, even those that appear to be cured by resection or irradiation of the tumor, are treated with intensive chemotherapy similar to that used to treat ANLL during the nonleukemic period as soon as possible.     

Germ cell tumors of the brainstem: report on two cases with pulmonary complications and a review of the literature

Intracranial germ cell tumors most commonly present in the pineal and suprasellar regions. Rare cases of brainstem germ cell tumors have been described. Here, we present two cases with brainstem germ cell tumors and review the current literature. The two patients did well, in terms of disease control, but have significant morbidity related to respiratory issues. One patient remains ventilator dependent and the second died from respiratory arrest.     

微囊型脑膜瘤2例临床病理学特征分析并文献复习

背景与目的：微囊型脑膜瘤是脑膜瘤的罕见亚型,国内外报道较为少见,且多以个案报道为主。本研究旨在探讨微囊型脑膜瘤的临床病理学特点及鉴别要点。方法：回顾分析2例微囊型脑膜瘤的临床表现、组织学形态及免疫组织化学表型,并复习相关文献。结果：2例均为中年患者,男性和女性各1例,男性患者因外伤引起脑出血就诊;女性患者因头痛就诊,病变分别位于左侧额部和右侧额部,最大径分别为5.75和5.47 cm。影像学改变均示“脑外肿瘤”。镜下瘤细胞排列疏松,形成大小不等的微囊,腔内含粉染之浆液,肿瘤细胞具有空泡状细胞质和细长的细胞质突起,典型的漩涡状结构和砂粒体少见。免疫组织化学示,波形蛋白（vimentin）、上皮膜抗原（epithelial membrane antigen,EMA）和孕激素受体（progesterone receptor,PR）均呈阳性表达,S-100、胶质纤维酸性蛋白（glial fibrillary acidic protein,GFAP）、CD34和肌酸激酶（creatine kinase,CK）均呈阴性表达,Ki-67阳性率达3%~5%。2例患者均行手术治疗,术后均随访观察,随访时间为5~6个月,截至2018年1月,2例患者一般情况良好,均未见肿瘤复发。结论：微囊型脑膜瘤为脑膜瘤的特殊亚型,依据病理组织学形态及免疫组织化学可明确诊断。目前治疗主要以手术治疗为主,预后良好。     

Primary bone lymphoma: a report of two cases and review of the literature

Primary bone lymphoma (PBL) is an uncommon tumor accounting for approximately 4-5% of extra nodal lymphoma and less than 1% of all non-Hodgkin's lymphoma. Disease may be complicated at presentation by pathological fracture or spinal cord compression. Diffuse large-B-cell lymphoma (DLBCL) accounts for the majority of cases of PBL. Owing to its rarity, only a few retrospective studies have been published addressing the prognosis and treatment of primary bone lymphoma. In this paper, we report our experience with two cases of PBL treated with chemotherapy and radiotherapy and review literature to elucidate the optimal treatment of primary bone lymphoma.     

肾上皮样血管平滑肌脂肪瘤2例及文献复习

  目的  探讨肾上皮样血管平滑肌脂肪瘤(epithelial angiomyolipoma, EAML)的临床病理特征, 进一步认识本疾病。  方法  回顾性研究2002年7月至2012年8月2例肾EAML患者的临床及病理资料并进行随访, 结合复习文献, 对其临床病理特点、生物学行为及预后进行分析。  结果  2例患者都为女性, 平均年龄47.5岁, 均为左肾EAML。无明显阳性体征, 2例在B超和CT均发现肾占位, 其中1例CT强化不均匀, 1例增强检查呈渐进性强化, 延迟期强化程度略减低。分别行左肾部分切除术和根治性左肾切除术。组织形态学的主要特征均呈明显的上皮样分化。  结论  分析结果提示: EAML生物学行为与经典型肾AML不同特征在于浸润性生长方式、细胞学异型性, CT对其诊断有重要意义, 但病理结合免疫组化能够确诊, 手术是主要的治疗方法, 其预后大多数良好。      

下颌骨成釉细胞瘤伴肺转移1例:病例报告并文献复习

目的:探讨成釉细胞瘤及恶性成釉细胞瘤的临床特征、诊断、治疗方法,以提高对该疾病的认识。方法:回顾性分析我科收治的1例下颌骨成釉细胞瘤肺转移患者的临床资料,并结合近些年国内外相关文献对该疾病进行回顾性分析。结果:本例患者肺部肿瘤切除术后一般情况良好,术后肺部肿瘤组织基因检测发现有KMT2D基因、BCOR基因、AKT1基因、BRAF基因突变。结论:下颌骨成釉细胞瘤伴肺转移临床上较为罕见,其确诊依靠病理结果。发生转移的机制和治疗方法还存在一定的争议,需要进一步探索。     

Investigation and diagnostic imaging of suspected pulmonary embolism during pregnancy and the puerperium: A review of the literature

Pulmonary embolism (PE) is a leading cause of maternal mortality with women at increased risk of PE during pregnancy and the early postpartum period. Clinical assessment of suspected PE during pregnancy is challenging as signs and symptoms associated with PE overlap with physiological changes of pregnancy. Clinical tests and rules commonly used to assess pre‐test probability of PE were historically not well validated in the pregnant population. The challenges of clinical assessment in the pregnant and postpartum population result in a lowered threshold for diagnostic imaging. Computed tomographic pulmonary angiography (CTPA) and nuclear medicine lung scintigraphy or ventilation/perfusion (V/Q) scans are the main types of diagnostic imaging for suspected PE. Both methods are associated with small levels of ionising radiation exposure to mother and foetus. Accuracy of the diagnostic imaging tests is paramount. Haemodynamic changes of pregnancy, including increased heart rate, increased blood volume and altered flow velocity in the pulmonary arteries, may influence the quality of imaging. This comprehensive review examines the literature and evidence for the investigation and diagnostic imaging of suspected pulmonary embolism during pregnancy with CTPA and V/Q. Clinical decision‐making tools, biomarkers and diagnostic imaging during pregnancy and postpartum will be considered with a focus on diagnostic accuracy and yield, radiation dose exposure (maternal–foetal) and protocol modifications. Current practice guideline recommendations and recent literature on diagnostic pathways are also presented.     

Carcinomas arising in fibroadenomas: a report of two cases and a review of the literature

Carcinomas arising in fibroadenomas are rare. Two such cases are being presented; one is of the ductal variety and the other, lobular. Both cases clearly demonstrate the overall configuration of fibroadenomas with foci of in situ and infiltrating carcinoma, the surrounding breast tissue being devoid of malignant changes. The diagnosis was clinically unsuspected in both cases and in only one of them was it suspected at the time of gross pathological examination. The intent of this presentation is to increase general awareness as to the existence of carcinomas arising in fibroadenomas and also to actively discourage the practice of rendering gross pathological diagnoses of fibroadenomas at table diagnosis, however innocuous these neoplasms may appear to be, without microscopic examination by frozen sections.     

Primary gastric T-cell lymphomas: report of two cases and a review of the literature

To understand more fully the clinicopathological features of primary gastric T-cell lymphomas (PGTL), we report two cases of PGTL and review the literature. The present cases were not associated with human T-cell leukemia virus type 1 (HTLV-1) and were at clinical stage IIE. In both cases, T-cell origin of the lymphoma cells was diagnosed immunohistochemically. The clinical courses of these two cases were different: one followed a very aggressive clinical course and the patient died 6 months after the diagnosis, whereas the other patient survived more than 2 years without adjuvant chemotherapy. Clinicopathological features of 23 patients with PGTL are summarized with regard to their differences from primary small intestinal T-cell lymphomas (PSITL) and by association with HTLV-1. The median age at onset of PGTL was 58 years. The gender ratio was male-dominant (M:F = 2.3:1). About two-thirds (10 of 17) of PGTL cases had evidence of HTLV-1 infection. The most common presenting symptom for PGTL was upper abdominal discomfort and/or pain (76%), whereas that in PSITL was weight loss (61%) and diarrhea (42%). Typical lesions for PGTL were large ulcerations at the corpus to antrum. Neoplastic cells had no typical morphological characteristics for PGTL including HTLV-1-associated cases. CD3+4+8- was the most frequently observed surface phenotype of PGTL cells. Laboratory findings at diagnosis were not informative. Most patients were treated by gastrectomy with or without chemotherapy. PGTL, excluding that with HTLV-1, showed better prognosis than PSITL, although PGTL with HTLV-1 had a poorer prognosis.     

以噬血细胞综合征为首发表现的非霍奇金淋巴瘤二例并文献复习

目的 探讨以噬血细胞综合征(HPS)为首发表现的非霍奇金淋巴瘤(NHL)的特点.方法 回顾性分析2例以HPS为首发表现的B细胞NHL患者的发病及治疗转归,并复习国内外文献.结果 2例患者均以HLH-94为基础的方案控制发热后,继以R-CHOP(地塞米松替代泼尼松)方案化疗反应良好.通过复习文献发现B细胞淋巴瘤相关噬血细胞综合征(B-LAHS)中主要淋巴瘤类型为弥漫大B细胞淋巴瘤,T细胞淋巴瘤相关噬血细胞综合征(T-LAHS)中淋巴瘤类型分布无显著差异.中国、韩国的T-LAHS发病率明显高于B-LAHS,在西方国家两者发病率无显著差异.T细胞淋巴瘤更易罹患HPS.B-LAHS患者中位年龄明显高于T-LAHS,中位生存时间和总生存率都优于T-LAHS.利妥昔单抗的应用为B-LAHS带来生存优势.结论 以HPS为首发表现的B细胞NHL应用依托泊苷联合R-CHOP方案可较快控制病情,后续疗效好.     

Cyclophosphamide-induced cardiomyopathy: a report of two cases and review of the English literature.

Fatal cardiomyopathy developed in two patients receiving cyclophosphamide in preparation for bone marrow transplantation. Both patients had normal EKGs prior to receiving cyclophosphamide in total doses of 168 mg/kg (case 1) and 144 mg/kg (case 2) and subsequently developed loss of voltage and ST-T wave changes. One patient (case 1) died of CHF and hypotension while the other patient (case 2) developed tamponade. Prior to this report, the lowest total dose of cyclophosphamide reported to cause fatal cardiomyopathy was 180 mg/kg. In contrast to anthracycline congestive cardiomyopathy, the effects of cyclophosphamide appear to have an acute onset and do not appear to be the cumulative result of drug dosing. Postmortem examination in both patients revealed thickened left ventricles with intramyocardial hemorrhage.     

Ossifying fibroma of the jaws: report of two cases and literature review

Ossifying fibromas are uncommon benign tumors of the craniofacial skeleton thought to originate from the periodontal ligament. Most are small and incidentally diagnosed with routine dental radiographs. With larger lesions, patients may complain of an abnormal bite or an enlarging mass. This tumor involves slow-evolving growth with deforming swelling generally arising in the mandible, with possible early tooth displacement. From the radiological perspective, more than 50% of the lesions exhibit an expansion of the jaws and 53% shows well-defined unilocular radiolucencies and 40% are mixed radiolucent-radiopaque lesions. The lesions exceptionally can be radiopaque. Ossifying fibroma presents several variant histopathological subtypes. The overlapping clinical and histopathological features of these subtypes have led to diagnostic dilemma and confusion. Complete excision of this tumor has become a necessity since it is notorious for recurrence. We present here two cases of ossifying fibroma of the jaws along with insight into the literature review.