[One Case of Accessory Breast Cancer Complicated by Contralateral Breast Cancer].

We experienced a case of right sided accessory breast cancer complicated by contralateral breast cancer. A 50-year-old woman came to us for an examination because a tumor in her left breast was pointed out at breast cancer screening. A breast MRI confirmed a tumor in her left breast and a tumor continuing from the skin to the subcutis of the right axilla. A skin biopsy for the tumor in the right axilla and a core needle biopsy(CNB)for the tumor in the left breast were performed. The pathological result of the CNB for the left breast indicated an invasive ductal carcinoma of the tubular formative scirrhous type. Although the tumor of the right axilla was poorly differentiated adenocarcinoma demonstrating cord-like arrays, it was examined by skin biopsy and therefore no deep part of the tissue was included. We conducted immunostaining, in consideration of the possibility of metastasis from the left sided breast cancer. ER, PgR, mammaglobin, GATA 3 were positive, strongly suggesting that the tumor in the right axilla was also derived from a mammary gland. We also performed a wide local excision of the right axilla plus axillary dissection(level Ⅰ)in addition to conducting a left mastectomy plus sentinel lymph node biopsy, in consideration of the possibility of primary right sided accessory breast cancer. The pathological result following surgery confirmed a difference in the histologic features between both sides, residual normal accessory mammary glands around the tumor on the right side, and the presence of rich DCIS and a lobular replacement image, leading to a definitive diagnosis of primary invasive ductal carcinoma of the accessory breast on the right side.

Establishment and characterization of a novel VEGF-producing HHV-8-unrelated PEL-like lymphoma cell line, OGU1.

Primary effusion lymphoma (PEL) is a rare B-cell lymphoma subtype that is characterized by lymphomatous effusion without the presence of masses, and it typically occurs in human immunodeficiency virus (HIV)-infected individuals. Lymphoma cells are universally positive for human herpesvirus 8 (HHV-8). Recently, a cavity-based effusion lymphoma that is similar to PEL without HHV-8 infection, called HHV-8-unrelated PEL-like lymphoma, has been reported in non-HIV-infected individuals. However, the pathophysiology of this lymphoma is largely undefined. We established a novel B-cell line OGU1 derived from a patient with HHV-8-unrelated PEL-like lymphoma. Notably, OGU1 cells produced vascular endothelial growth factor (VEGF) and expressed VEGF receptor 1, whose inhibitors retarded cell growth. Because VEGF acts as a vascular permeability and growth factor, it could play a role, at least in part, in the pathogenesis of this unique lymphoma. Thus, the OGU1 cell line is useful for the investigation of HHV-8-unrelated PEL-like lymphoma.

[Two Cases of Primary Intracranial Solitary Fibrous Tumor:Genetic Examination of NAB2-STAT6 Fusion and Its Association with Hemangiopericytoma].

Using whole exome and transcriptome sequencing, NAB2-STAT6 gene fusions have recently been identified in patients with solitary fibrous tumors (SFT). We report two cases of SFT in which NAB2-STAT6 fusions were identified. The patients were a 32-year-old man with a parasagittal tumor involving the superior sagittal sinus, and a 40-year-old man with a cerebellar convexity tumor partially involving the transverse sinus. Their tumors were gross totally resected and diagnosed to be SFT according to the following pathological findings:the tumors were composed of spindle cells with a patternless architecture, with prominent stromal collagen and staghorn vessels. An immunohistochemical study yielded positive results for CD34, CD99, and Bcl-2 and negative results for EMA, GFAP, and S100. The MIB-1 indexes were 13 and 7%, respectively. NAB2-STAT6 fusions were detected in both cases with a common fusion variant, NAB2ex6-STAT6ex16/17. We also identified NAB2-STAT6 fusions in two hemangiopericytomas diagnosed in the past with a common variant of NAB2ex6-STAT6ex16/17. These findings suggest that solitary fibrous tumor and hemangiopericytoma may be diagnosed based on the presence of NAB2-STAT6 fusion, and not classified separately because of the same genetic background.

[Basaloid Carcinoma of the Esophagus with Lugol-Voiding Lesions--A Case Report].

Patients with Lugol-voiding lesions of the esophagus are frequently affected with multiple cancers. Basaloid carcinoma of the esophagus is a very rare disease characterized by growth in the submucosal layer that exhibits a submucosal tumor-like shape. There have been some reports that this type of carcinoma is biologically high-grade. We report a case of metachronous squamous cell carcinoma in situ and superficial basaloid carcinoma in the esophagus with Lugol-voiding lesions. A 63-year-old man underwent gastrectomy for gastric cancer at the age of 45 years. The subsequent surveillance endoscopy found a type 0-Ⅱc lesion in the esophagus in 2000. EMR was thus performed. The pathology showed squamous cell carcinoma in situ. Dysplasia was diagnosed based on the Lugol-voiding lesions, and EMR was performed twice. In 2014, a fourth EMR was performed after a high-grade intraepithelial neoplasia was diagnosed. The pathology showed squamous cell carcinoma in situ and a basaloid carcinoma in the lamina propria, T1a-LPM, ly0, v0, pHMX, pVM0. The patient has had no recurrence for approximately 1 year after the fourth EMR.

[A case of cavernous sinus epidermoid: anatomical classification and surgical approach].

Epidermoid rarely appears in the cavernous sinus. We encountered a 41-year-old man with left abducens nerve palsy. A round-shaped, low-density lesion on CT was located lateral to the left cavernous sinus with a compressed and thinned lateral wall of the sphenoid sinus. We could not identify cranial nerves in the cavernous sinus, which was found to be packed with a non-enhanced, high-intensity tumor on both T1 and T2 MRI. Part of the tumor capsule and its pearly contents were removed with an intradural subtemporal approach, and an inner membranous layer with cranial nerves and a tumor capsule were seen at the bottom of the tumor cavity. Postoperatively, complete cure was achieved. Reviewing similar cases, we found 18 cavernous sinus epidermoids:extracavernous type in 5;interdural in 10;and intracavernous in 3. The interdural type could be further divided into two subtypes:superficial cavernous sinus and inner membranous types. The present case belongs to the former. Frontotemporal and subtemporal procedures via both intra- and extradural approaches are relatively safe for lesions except for the intracavernous type, because cranial nerves are not located in the lateral wall of the tumor. MRI provides more useful information on cranial nerves and aid in choosing a better treatment strategy.

[Trigeminal neuralgia caused by cerebellopontine angle lipoma:a case report and review of the literature].

A 59-year-old man presented with right trigeminal neuralgia of the second branch, which had been treated with carbamazepine. The pain could not be controlled adequately because of side effects. CT and MRI revealed a 2-cm lesion in the right cerebellopontine angle. Retrosigmoid lateral suboccipital craniectomy was performed, and a soft yellowish mass was found to be associated with the 5th, 7th, and 8th cranial nerves, anterior inferior cerebellar artery, and small vessels. The lipoma was partially resected from around the root entry zone(REZ)of the 5th nerve and small vessels were coagulated around the REZ. After surgery, there was no trigeminal neuralgia, but facial numbness and cerebellar signs were noted. Postoperative MRI showed decompression of the trigeminal nerve and venous infarction in the middle cerebellar peduncle. Reviewing similar cases, we found 19 lipoma patients presenting with trigeminal neuralgia. Symptoms of involvement of other cranial nerves were also present in 11 patients, and 14 were younger than 30 years old. Of 17 surgical cases, total resection was not attempted apart from one case. Although pain relief was achieved in all surgical cases, complications developed in 11. Surgery should be performed only in patients with disabling and uncontrolled symptoms.

[Successful treatment with rituximab in a patient with splenic marginal zone B-cell lymphoma accompanied by cold agglutinin disease].

An 81-year-old man was admitted to our hospital due to dyspnea in July 2008. A physical examination revealed marked splenomegaly, and the results of laboratory tests were as follows: hemoglobin (Hb)=7.0 g/dL, Ret=6.4%, WBC=24,100/µL (Ly: 20,003/µL), indirect bilirubin=3.6 mg/dL, LDH=232 IU/L. The cold agglutinin titer was 1 : 8,192, and a direct antiglobulin test was positive. A PET scan showed abnormal accumulation in the spleen and bone marrow. A bone marrow aspirate examination and biopsy demonstrated diffuse involvement of abnormal lymphocytes that were found to be positive for CD20 and negative for CD5, CD10, and cyclin D1. The immunoglobulin genes were clonally rearranged. Based on these findings, splenic marginal zone B-cell lymphoma (SMZL) associated with cold agglutinin disease (CAD) was diagnosed. Because the patient refused splenectomy, he was treated with four cycles of rituximab therapy (375 mg/kg, once a week). The Hb level and lymphocyte count subsequently normalized and the splenomegaly resolved. One year later, he relapsed and was again treated with rituximab therapy with complete remission. CAD accompanied by SMZL is very rare. Rituximab may be chosen as an alternative and effective therapeutic option in patients with SMZL-particularly those with autoimmune hemolytic anemia.

[Pseudosarcomatous myofibroblastic proliferation of the bladder-case report].

A 66-year-old man with a history of sporadic von Recklinghausen' s disease was treated for pseudosarcomatous myofibroblastic proliferation in the bladder. The patient had a history of repetitive recurrence of bladder carcinoma requiring transurethral resection (TUR) 4 times and 1 course of intravesical BCG instillation. Three months after the fourth TUR, an intravesical solid mass 4 cm in diameter was detected. Suspicion of recurrent bladder carcinoma led to a fifth TUR procedure. Pathological findings showed submucosal growth of myofibroblasts with myxoidal stroma and we made a diagnosis of Pseudosarcomatous myofibroblastic proliferation. The patient was well with no evidence of recurrence after 1 year.

A comparison of indocyanine green fluorescence imaging plus blue dye and blue dye alone for sentinel node navigation surgery in breast cancer patients.

PURPOSE: To evaluate two methods of sentinel node navigation surgery (SNNS) using blue dye with and without indocyanine green (ICG) fluorescence imaging (FI) to determine the usefulness of combined ICG and blue dye. METHODS: Between 2005 and 2010, a total of 501 patients underwent SNNS in our hospital. Detection of sentinel lymph node (SLN) was performed with sulfan blue (SB) alone until 2008 and with a combination of SB and ICG-FI since 2009. ICG 5 mg and SB 15 mg were injected in the subareolar region, and FI was obtained by a fluorescence imaging device. RESULTS: We attempted to identify SLNs in 393 patients by SB alone and in 108 patients by a combination of SB and FI. The mean number of SLNs detected was 1.6 (0-5) for SB alone and 2.2 (1-6) for the combination method. The SLN identification rate was 95.7 % for SB alone and 100 % for the combination method so that the combination was significantly superior to SB in terms of the identification rate (p = 0.0037). In patients who received the combination method, detection of SLN was made through only SB in 1 patient, only ICG in 8 patients, and both in 99 patients. Lymph node metastasis was found in 56 patients with SB alone and in 16 patients with the combination method. Recurrence of an axillary node was observed in 3 patients (0.8 %) with SB alone and in no patients with the combination method. CONCLUSIONS: ICG-FI is a useful method and is especially recommended in cases where no radiotracers are available.

[Renal epithelioid angiomyolipoma with malignant behavior].

The patient was a 22-year-old woman with a history of tuberous sclerosis who complained of abdominal distention. Computed tomography showed bilateral renal masses with minimal fat tissue that were unlike classical angiomyolipoma (AML), as well as enlargement of the paraaortic lymph nodes, pleural effusion, and ascites, similar to other advanced malignant neoplasms such as renal cell carcinoma and renal sarcoma. We performed a left nephrectomy to relieve the symptoms. A pathological examination revealed that the tumor was composed of polygonal epithelioid cells positive for both αSMA and HMB-45, as well as spindle-shaped cells, while immunoreactivity proved the tumor to be an eAML, a subtype of AML with malignant potential. Although the patient was scheduled for postoperative adjuvant chemotherapy, her condition worsened and she died on postoperative day 24 before receiving chemotherapy. We discuss this case with reference to previous reports.

[Responses to primary systemic therapy for breast cancer as assessed by hormone receptor and HER2 status].

The aim of this study was to investigate responses to primary systemic therapy (PST) for breast cancer, by hormone receptor (HR) and HER2 status. This study included 107 women with T>3 cm and/or node-positive breast cancer who received PST at this department between March 2004 and January 2009. Treatment with epirubicin and cyclophosphamide (EC) followed by docetaxel (DTX) therapy was undertaken up to December 2005. From January 2006, EC followed by weekly paclitaxel (PTX) with or without trastuzumab (T) therapy was performed. From February 2008 and thereafter, the EC-PTX-T therapy was continued in HER2-positive patients, whereas the preceding EC-DTX therapy was administered in HER2-negative patients. Clinical responses of the 107 patients (56 were treated with EC-DTX, 37 with EC-PTX, and 14 with EC-PTX-T) were as follows: CR was achieved in 18 patients, PR in 74 patients, SD in 12 patients, and PD in 3 patients, with a response rate of 86. 0%. Histologically, 14 patients(13. 2%)had pathological CR(pCR)in a limited sense. When these patients were further divided according to HR status, those positive for both estrogen receptor (ER) and progesterone receptor (PgR) accounted for 1. 8%, those positive for ER and negative for PgR accounted for 5. 3%, and those negative for both ER and PgR accounted for a significantly higher percentage of 40. 0% (p<0. 0001) . By HER2 status, pCR was achieved at a significantly higher rate (47. 8%) of HER2-positive patients, compared to 3. 6% of HER2-negative patients (p<0. 0001). Common adverse events included Grade 3/4 leukopenia (57. 9%), neutropenia (67. 3%), and Grade 3 febrile neutropenia (11. 2%). The results show that a higher pCR rate can be expected after PST in HR-negative patients and HER2-positive patients. HER2-positive patients would particularly benefit from preoperative anthracycline chemotherapy followed by a taxane combined with trastuzumab.

[A Case of Liver Dysfunction Requiring Hospital Admission after Taking Oral Itraconazole for the Treatment of Kerion Celsi].

67-year-old female patient developed drug-induced liver dysfunction after taking oral itraconazole (ITCZ) for the treatment of kerion celsi. Red papules appeared on the temporal area of the patient one month prior to her visit to our clinic. The patient presented with a nodule with yellow crust, erosion, infiltration, and hair loss on the area. Diagnosis of kerion celsi caused by Trichophyton rubrum was made from clinical, pathological, and mycological findings. Laboratory data showed normal liver function, and the patient was not taking any other medication, thus, daily oral ITCZ 100 mg was started. The skin lesion improved, but severe liver dysfunction was found 1 month after starting ITCZ. Oral ITCZ was therefore terminated, and the patient was admitted to a medical ward for the treatment of liver dysfunction. Hepatobiliary enzymes increased after admission: AST 232 IU/L, ALT 465 IU/L, T-bil 6.1 mg/dL, and D-bil 3.9 mg/dL. The patient was kept at rest and was given oral ursodeoxycholic acid. Hepatobiliary enzymes returned to normal level 2 1/2 months after starting ITCZ. The skin lesion healed without further treatment. No recurrence was observed. It is noteworthy that liver function has to be carefully monitored during treatment with oral ITCZ.

Chronology of lichen planus-like keratosis features by dermoscopy: a summary of 17 cases.

Dermoscopic findings for 17 cases of lichen planus-like keratosis (LPLK) were chronologically evaluated. Three males and 14 females were included in the study and the ages ranged from 43 to 85 years (median 65 years). Three cases were diagnosed based on stereotypical dermoscopic findings, while the other 14 cases were histopathologically diagnosed as LPLK. Dermoscopy photographs were divided into four groups depending on the number of days (D) from the initial visit: 1) D = 0 (initial visit or biopsy day); 2) D = 61 to 180; 3) D = 181 to 270; 4) D = 271 to 360. Dermoscopic findings, described as light brown pseudonetwork, pinkish area, gray pseudonetwork, annular granular structures, and blue-gray fine dots, were evaluated at every visit to the hospital. Initial dermoscopy features included light brown pseudonetworks due to residual solar lentigo and overlapping pinkish areas attributed to lichenoid inflammation. Annular granular structures and gray pseudonetwork appeared to be the main features of the regressing stage; these features seemed to progress to "blue-gray fine dots" in the late regressing stage. Blue-gray dots or globules reflecting melanophages, the hallmark dermoscopic features of LPLK, were believed to resolve in approximately one to two years. Based on the clinical and dermoscopic observations, we have specified five stages of evolution of LPLK, namely 1) pre-existing solar lentigo, 2) early inflammatory stage, 3) early regressing stage, 4) regressing stage, and 5) late regressing stage. The limitations of the study are that this is a small-sized, retrospective, observational study and that ethnicity of participants is limited to Japanese patients with skin phototype III.

A case of cervical esophageal duplication cyst in a newborn infant.

Esophageal duplication cyst is a rare congenital anomaly resulting from a foregut budding error during the fourth to sixth week of embryonic development. Cervical esophageal duplication cysts are very rare and may cause respiratory distress in infancy. A full-term newborn girl who was born by normal delivery was transferred to our hospital because of swelling of the right anterior neck since birth. Cervical ultrasonography showed a 40 × 24 × 33 mm simple cyst on the right neck. Tracheal intubation was required at 2 weeks of age because of worsening external compression of the trachea. Fine-needle aspiration cytology revealed the existence of ciliated epithelium. At 1 month of age, exploration was performed through a transverse neck incision. The cyst had a layer of muscle connected to the lateral wall of the esophagus. Histopathological diagnosis was a cervical esophageal duplication cyst. We describe the clinical features of infantile cervical esophageal duplication cysts based on our experience of this rare disease in a neonate, along with a review of 19 cases previously reported in literature.

Condylomata lata on the ankle: an unusual location.

A 43-year-old Japanese man presented with reddish nodules on the ankle. The nodules had a yellowish crust and eroded surface. Dermoscopy revealed red to milky-red globules at the periphery and some glomerular vessels in the center and a whitish-pink network, which corresponded to capillary dilatation in the papillary dermis and prominent acanthosis, respectively. These structures were surrounded by a yellowish peripheral structureless area and multiple white, small, round structures in the center, corresponding to the macerated horny layer and keratin plugs. Blood samples were positive for rapid plasma reagin (1:64), Treponema pallidum hemagglutination assay (1:20480), and fluorescent treponemal antibody-absorption (1:1280). A lesional skin biopsy specimen showed irregular acanthosis and papillomatosis. The Warthin-Starry and anti-Treponema pallidum antibody stains on the biopsy specimen revealed many spirochetes in the lower epidermis and the papillary dermis. A diagnosis of secondary syphilis with condylomata lata was made. After one week of treatment with oral benzylpenicillin benzathine hydrate (Bicillin(®) G granules 400,000 units; Banyu Pharmaceutical Co., Ltd, Tokyo, Japan), 1.6 million units (U) daily, the ankle lesions had resolved with a small ulcer and pigmentation. Although syphilis is a relatively common disease, this case study reports an unusual presentation as well as dermoscopy findings.

Histopathological diagnosis and prognostic factors in adrenocortical carcinoma.

A great majority of adrenocortical tumors are benign, and many adrenocortical carcinomas (ACC) are obviously malignant at presentation. The histopathological diagnosis of ACC is occasionally difficult, particularly with stage I and stage II disease. The prognosis of ACC is generally poor. Surgery is the major treatment, with chemotherapy and radiotherapy being applicable to only restricted patients. The Weiss criteria are useful in diagnosing the common adult type of ACC. Histopathological prognostic factors of ACC have not been fully established because of the rarity of the disease. In this article, we first describe the current histopathological diagnostic and prognostic factors of ACC, highlighting the special types of ACC to which Weiss's criteria are not fully applicable. These special type tumors include pediatric adrenocortical tumors, oncocytomas, and aldosterone-producing tumors of pure zona glomerulosa type. Then we present three cases with unusual small adrenocortical tumors. One patient had an unequivocal ACC showing metastatic disease. One had a histologically defined ACC with no metastasis or macroscopic invasion. The third was a pediatric patient with a tumor showing a nodule-in-nodule pattern with insulin-like growth factor II expression.

Abrupt Intralesional Color Change on Dermoscopy as a New Indicator of Early Superficial Spreading Melanoma in a Japanese Woman.

Diagnosis of superficial spreading melanoma in the early stage is often difficult, even with dermoscopy. We report the case of a 37-year-old Japanese woman with superficial spreading melanoma in her left buttock. The lesion developed 20 years before becoming visible and gradually enlarged over the past few years without any symptoms. Physical examination showed a well-demarcated dark-brown macule 10 mm in diameter. Dermoscopy demonstrated a central dark area with a blue-grey structureless area, a milky-red area with irregular blue-grey dots or globules suggestive of regression structures, and multifocal black pigmentation with whitish scaly areas. An abrupt intralesional change in color from a central dark area to a peripheral light-brown area was also seen. The peripheral area showed an atypical pigment network with an obscure mesh and holes. Histopathologic examination of the lesion showed acanthosis with melanocytic proliferation and nuclear atypia, a band-like lymphocytic infiltrate, melanophages and a few nests of melanocytes just beneath the epidermis. The epidermal melanocytes were positive for S-100, Melan-A and HMB-45, but the dermal nests of melanocytes were negative for HMB-45 and positive for S-100 and Melan-A. A diagnosis of superficial spreading melanoma with a tumor thickness of 0.4 mm (pT1aN0M0, stage 1A) was established based on the clinical, dermoscopic and histopathologic findings. This case suggests that dermoscopy is useful in the diagnosis of this condition. An abrupt intralesional change of color might be a new indicator of early superficial spreading melanoma.

Calcinosis cutis confined to the dermis after intravenous administration of a calcium preparation: a case report and review of the Japanese literature.

A 61-year-old female received intravenous injection of calcium chloride after common iliac artery bypass surgery. A red flare appeared at the site of the intravenous infusion on the left forearm and gradually progressed to induration. Seven weeks later, she was referred to the Department of Dermatology for management. Physical examination showed an indurated plaque measuring 13 × 65 mm in size, with linearly distributed ulcers covered by yellowish-white substance, surrounded by reddish skin. Laboratory tests showed no significant abnormalities including serum calcium, phosphate and thyroid hormones. Cultures were negative for microorganisms. Histopathological examination showed calcium deposition confined to the dermis. The lesion healed spontaneously within 2 months with scar formation. A review of the Japanese literature showed confinement of calcium deposits to the dermis in most of the reported cases. We speculate that the pathomechanism of dermal calcinosis includes needle-induced tissue injury with capillary destruction, leading to release of excess calcium between collagen fibers, and its binding to phosphate in the dermis and deposition as calcium phosphate crystals.