Toshio Oyama

Simultaneous Bilateral Spontaneous Pneumothorax Secondary to Metastatic Angiosarcoma of the Scalp: Report of a Case

Angiosarcoma is a highly malignant neoplasm, which most often develops on the scalp or face of elderly people. Common distant metastatic sites include the lung, liver, lymph nodes, and skin. We report a case of angiosarcoma manifesting as simultaneous bilateral spontaneous pneumothorax secondary to pulmonary metastases in an 86-year-old man. The pneumothorax preceded the diagnosis of angiosarcoma. Chest computed tomography showed multiple thin-walled cavitary metastatic pulmonary lesions, which increased in size as new lesions appeared over the clinical course of several months. This case suggests that a finding of simultaneous bilateral spontaneous pneumothorax may indicate a serious parenchymal lung disorder.

The Effects of Augmented Hemodynamic Forces on the Progression and Topography of Atherosclerotic Plaques

In order to clarify mechanisms determining different degrees of vulnerability of atherogenesis between the apical and the proximal lateral walls at branchings, both regions of the inferior mesenteric artery in human autopsy cases were investigated electron microscopically. The lateral wall and the apex have been accepted by many researchers as the most preferential and the most resistant sites, respectively, for the disease. In regard to blood flow, the apex is exposed to laminar high shear stress, but the outer lateral wall to turbulent low shear stress. In newborns, intimal thickness in the apex was greater than that in the lateral wall, due mainly to the proliferation of SMC. After the 3rd decade, collagen fibers drastically increased in the apical intima, and SMC embedded between the collagen fibers, modulating their phenotypes from synthetic to contractile. In the lateral intima, SMC remained as the synthetic type. Synthetic SMC are considered capable of proliferation in the arterial wall. The lateral intima was generally abundant in proteoglycans and lacked collagen (including subendothelial basement membranes) as well as elastic fibers, particularly in the upper part of the intima. Such a structural difference may cause favorable conditions for atherosclerosis. Results of in vitro studies revealed that collagen gel suppressed proliferation of SMC and changed their phenotype from synthetic to contractile. Therefore, laminar high shear stress gives the arterial wall resistancy to atherogenesis through this phenotypic change. Rabbits showed preferential regions in certain areas of the flow divider for lipid deposition which were different from those of human beings. These regions were covered by ellipsoidal endothelial cells, which should be exposed to relatively low mean shear stress. Ellipsoidal endothelial cells had already been observed in intact rabbits. Therefore, we can conclude that atherogenic processes could be initiated by relatively low mean shear stress in either humans or rabbits.

Adenocarcinoma Arising in Anterior Mediastinal Mature Cystic Teratoma : Report of a Case

Mediastinal mature teratoma with malignant components is a very rare condition. A 57-year-old man presented with a large (14 × 10 cm) encapsulated and lobulated cystic mass involving the anterior mediastinum. The most consistent diagnosis on the basis of radiological findings was mature cystic teratoma, and a surgical resection was thus indicated. The operation was performed through a median sternotomy, and the tumor was extirpated with a combined resection of the pericardium and left mediastinal pleura. Although the pathological diagnosis was mature cystic teratoma, this patient showed a recurrence of malignant effusion 7 months following the operation. The earlier resected specimen was pathologically re-reviewed according to the blocked entire mass, and small foci of adenocarcinoma were thus found within the tumor.

High prevalence of the MYD88 mutation in testicular lymphoma: Immunohistochemical and genetic analyses

The activating mutation of MYD88 has been identified in diffuse large B‐cell lymphoma (DLBCL). We investigated the mutational status and both the gene amplification and protein expression of MYD88 in 23 cases of testicular DLBCL. To detect the MYD88 mutations, we employed the allele‐specific PCR and Sanger sequencing. MYD88 gene amplification and protein expression were analyzed by quantitative PCR and by immunohistochemistry, respectively. There were 17 cases of primary testicular DLBCL: 94% (16/17) exhibited a non‐Germinal center B‐cell (non‐GCB) subtype, 82% (14/17) showed the MYD88 L265P, and 65% (11/17) had intense expression of MYD88. When compared with normal lymph nodes, the MYD88 is significantly amplified in primary testicular DLBCL. However, the amplification status showed no correlation with its mutational status or protein expression. Moreover, neither the MYD88 mutational status nor the expression pattern affected overall survival. Six cases were secondary testicular DLBCL with an 83% (5/6) and an 80% (4/5) incidence of the non‐GCB subtype and of the MYD88 L265P, respectively. In conclusion, we demonstrated a high prevalence of the non‐GCB subtype and the common MYD88 L265P in both primary and secondary testicular DLBCL. Our data suggest that the MYD88 mutation is a fairly consistent genetic feature in testicular DLBCL.

Prognostic and therapeutic implications of the MIB-1 labeling index in breast cancer

BackgroundAssessment of tumor proliferative activity is considered to be the most powerful prognostic factor aside from axillary lymph node status. The purpose of this study is to assess the clinical value of measurement of proliferative activity using the MIB-1 labeling index in patients with breast cancer.MethodsSurgical specimens from 36 patients with benign breast disorders and 146 patients with breast cancer were investigated. The MIB-1 labeling index was determined on the specimens stained by immunohistochemical methods as much as possible. Clinical factors associated with the MIB-1 labeling index were reviewed.ResultsThe MIB-1 labeling index for non-proliferative disorders, proliferative disorders, and breast cancer was 3.4±1.9%, 8.9±6.2% and 20±12%, respectively. The MIB-1 labeling index and tumor size, lymph node metastasis status, and clinical stage according to the TNM classification correlated significantly. Survival rate was inversely correlated with the MIB-1 labeling index. No patient with an MIB-1 labeling index of less than 10% had lymph node metastases, and all are alive without recurrence. Patients with an MIB-1 labeling index of over 30% had an extremely poor prognosis.ConclusionsThe MIB-1 labeling index is very useful for predicting both either extremely good or extremely poor prognosis, and axillary lymph node metastasis.

Well-differentiated neuroendocrine tumour of the breast showing peculiar endovascular spread.

Sir: Spread of an intravascular tumour embolus from a primary lesion can be found in some cancers: intraportal spread of hepatocellular carcinoma, postcaval spread of renal cell carcinoma, and so on. However, to the best of our knowledge, such a finding in breast cancers has not previously been reported in the English literature. The WHO classifies mammary carcinomas with neuroendocrine (NE) features as a special tumour entity, representing <1% of invasive breast carcinomas, and recognises three subtypes: (i) NE tumor (NET), well differentiated; (ii) NE carcinoma, poorly differentiated; and (iii) invasive carcinoma with NE differentiation. Herein, we describe the first case of a well-differentiated mammary NET with extensive intravenous spread. The patient, a 42-year-old premenopausal Thai woman, presented with a palpable mass in the subareolar portion of the left breast. Ultrasonography revealed a sharply marginated, hypoechoic left breast tumour showing heterogeneous internal echoes, with a cranially extending lumen-like structure filled with solid tumour with similar echoic findings (Figure 1A). On MRI, these lesions appeared as an oval-shaped mass and a continuous intravenous occupying lesion, respectively, both of which were strongly enhanced during the early phases of the dynamic study (Figure 1B). These images suggested an invasive cancer with a tumour thrombus. Systemic CT and bone scintigraphy detected no other suspicious lesions. Ultrasound-guided, fine needle aspiration of the breast mass yielded a cytological diagnosis of carcinoma. The cut surface of the lumpectomy specimen contained well-circumscribed, mixed brownish-red and grey-whitish, solid tumour nodules, measuring up to 17 9 15 mm in size. Histopathologically, the tumour was composed of a solid invasive growth of carcinoma cells with a peripheral palisading arrangement and a highly vascular stroma (Figure 2A,B). Haemorrhage was marked in the lesions. Carcinoma cells were polygonal or, occasionally, spindle-shaped with finely granular, slightly eosinophilic cytoplasm (Figure 2B). Their nuclei had ovoid or irregular shapes, a finely granular chromatin pattern, and small nucleoli. Mitotic figures were seen in seven of 10 high-power fields. An in-situ component composed of carcinoma cells with the same histological features was locally observed near the invasive cancer nests (Figure 2C). Massive tumour embolization within prominently dilated veins, spreading from the primary focus, was confirmed by the elastic Van Gieson method (Figure 2A,D). Focal lymphatic permeation

Progressing subglottic and tracheobronchial stenosis in a patient with CHARGE syndrome diagnosed in adulthood

A 33-year-old woman was admitted for a pseudocroup-like cough and wheezing after general anesthesia. Several months ago, she had undergone cardiac re-operation and turbinectomy, both of which had involved difficult intubations. Bronchoscopy indicated a pin-hall-like subglottic stenosis; therefore, emergency tracheotomy was performed. Six years later, a computed tomography scan demonstrated progressive stenosis of the entire circumference of the trachea and main bronchi. She died at 40 years. Her autopsy revealed marked tracheobronchial stenosis. She had many medical histories that had gone undiagnosed and had been clinically ill with only heart defects. She did not have coloboma but had microphthalmos, atresia choanae, retarded growth development, and deafness; thus, we diagnosed CHARGE syndrome that refers to multiple congenital anomalies, including airway abnormalities, which can lead to secondary complications such as traumatic stenosis after intubation. Physicians should have knowledge of this rare disease and should pay special attention to potential airway problems.

Tracheoesophageal Fistula Closed by Chemoradiotherapy in Lung Cancer

A 45-year-old man complaining of cough, dyspnea, and difficulty in swallowing was referred to our hospital. Chest CT scan showed a mediastinal mass compressing the trachea. He was diagnosed with poorly differentiated lung carcinoma by percutaneous needle biopsy. Bronchoscopy and upper gastrointestinal endoscopy revealed a tracheoesophageal fistula (TEF). Long-lasting febrile neutropenia made it impossible to continue chemotherapy, but a course of radiotherapy (total 61 Gy) was completed. The next endoscopy revealed closure of the TEF. Chemoradiotherapy (CRT) has been reported to close TEF in esophageal cancer, but the risk of a CRT-induced worsening of the fistula has dissuaded physicians from using CRT to treat TEF in lung cancer patients. CRT may serve as a palliative treatment for TEF in lung cancer as well as esophageal cancer.

Breast schistosomiasis japonica – a report of four Japanese cases

a history of locally advanced invasive ductal carcinoma of the left breast treated with neoadjuvant chemotherapy and excisional biopsy. There was a complete pathological response with absence of invasive carcinoma, changes consistent with chemotherapy effect, focal atypical ductal hyperplasia, radial scar and microcalcifications. Incidentally, a lobule with salivary gland-like acinar differentiation was noted in a background of benign breast tissue (Figure 1). The cells had slightly granular, amphophilic cytoplasm with zymogen-like secretory granules, a characteristic feature of serous acinar cells. These granules were readily highlighted by periodic acid–Schiff reaction (Figure 1). Breast neoplasms which exhibit eosinophilic and granular cytoplasm are varied, and acinic cell-like breast carcinoma can be included in this category. However, these tumours exhibit the features of malignancy with invasive growth, necrosis and cytological atypia, features absent in our case. Furthermore, the pretreatment core biopsy was invasive mammary carcinoma, no special type, without salivary acinar-like features. Changes of the breast resembling salivary gland have been reported in two cases, one associated with invasive ductal papillary carcinoma and the other with usual intraductal hyperplasia. Its identification raises the question of whether breast tumours with salivary gland differentiation originate from malignant transformation of terminal duct-lobular units with metaplastic changes. Alternatively, this type of salivary gland-like metaplasia might be part of the spectrum of histological changes seen in the breast after chemotherapy, or may merely be another form of metaplasia that has not been described in benign breast tissue. Finally, there is a possibility that this case represents a heterotopic salivary gland, as has been described in the neck, pituitary, parathyroid, mediastinum and rectum. Documenting these types of changes in various settings may be helpful in further defining the circumstances under which they occur and determining their significance, if any.

Spontaneous hemothorax caused by intrathoracic synovial sarcoma.

Synovial sarcoma, which is a soft tissue malignancy, primarily affects the extremities in the para-articular regions in adolescents and young adults. Synovial sarcoma of the pleural cavity is extremely uncommon, and there have been only a few reports in the literature. We report here an unusual case of left-sided spontaneous hemothorax as a presenting manifestation of intrathoracic synovial sarcoma in a 33-year-old pregnant woman. Spontaneous hemothorax, unrelated to trauma, is a very unusual clinical presentation, but we must consider entities with a physical risk assessment promptly because of the possible need for urgent management based on the hemodynamic condition of the patient. We should always be aware of possible causes of spontaneous hemothorax.