[Show abstract][Hide abstract] ABSTRACT: Low-grade endometrial stromal sarcoma is very rare and difficult to diagnose in the early stage. A standard treatment has not been established. In this case report of a patient with long-term survival, we describe an effective treatment for advanced low-grade endometrial stromal sarcoma.
A 24-year-old Japanese woman who presented with prolonged menstruation was diagnosed with leiomyoma on the basis of a specimen resected transvaginally. She underwent ten resections in 10 years without a malignancy diagnosis. During this period, she gave birth. At age 34 years, she visited our hospital, complaining of lower abdominal pain. A 10cm tumor was detected behind her uterus. The disease was diagnosed as an advanced malignant ovarian tumor before surgery. A laparotomy was performed, with many remnants left in the abdominal cavity. The final diagnosis was advanced low-grade endometrial stromal sarcoma. After 12 cycles of gemcitabine and docetaxel combination chemotherapy, the tumor disappeared completely. A retrospective pathological review of the specimens resected transvaginally showed that the tumors included low-grade endometrial stromal sarcoma elements. When the patient was age 42 years, the sarcoma recurred. It was detected around the right diaphragm and liver. Despite administration of gemcitabine and docetaxel, ascites and pleural effusion accumulated. Administration of medroxyprogesterone acetate, leuprorelin acetate, and anastrozole gradually reduced the ascites and pleural effusion. In addition to the three hormone drugs, 18 cycles of paclitaxel and carboplatin were administered. The patient recovered from her critically ill state and is currently alive with reduced tumor at age 45 years.
Our patient with low-grade endometrial stromal sarcoma whose disease began in her youth gave birth and experienced long-term survival with surgery, chemotherapy, and hormone therapy.
Preview · Article · Dec 2015 · Journal of Medical Case Reports
[Show abstract][Hide abstract] ABSTRACT: Granular cell tumors are uncommon, usually benign tumors of Schwann cell origin. The malignant variant is extremely rare, representing <2% of all granular cell tumors. Therefore, standard systemic chemotherapy for this disease does not exist. The present study reports the case of a 40-year-old female with a malignant granular cell tumor that originally arose in the right orbit and subsequently relapsed. The patient was started on pazopanib monotherapy following treatment with two investigational drugs, a smoothened inhibitor and then a phosphatidylinositol 3-kinase inhibitor, as part of a clinical trial. Although additional radiotherapy for local control was necessary, the lung metastases remained stable during the pazopanib monotherapy, which lasted for 7 months, following which a clinically stable disease state was determined. This case suggests that pazopanib can be a treatment option for the stabilization of disease progression in metastatic malignant granular cell tumor.
[Show abstract][Hide abstract] ABSTRACT: Breast cancer is occasionally complicated by sclerosing adenosis (SA). Although both lesions usually originate in the terminal duct lobular unit, their pathogenetic relationship has not yet been elucidated. The present study analyzed 63 breast cancer patients with SA (involving a total of 75 breasts) to clarify if coexisting SA increased the frequency of multicentric breast cancer or not. Using the topographical classification proposed in our previous study, breast cancers with SA were classified into the following three types: type A (n = 22), cancer area was completely surrounded by the SA; type B (n = 26), cancer area partially overlapped the SA; and type C (n = 27), cancer area was located separate from the SA. Breast cancers with SA had a significant (P < 0.001) increase in frequency of harboring bilateral and multicentric cancers [17 of 63 (27 %) and 15 of 63 (24 %), respectively] when compared to breast cancer patients without SA, regardless of topographical type. Breast cancers with SA were less invasive (P < 0.001), of lower histological grade (P = 0.034), and had similar frequency of estrogen receptor-positive (P = 0.21) and HER2-positive (P = 0.74) tumors. In conclusion, contralateral and ipsilateral multicentric breast cancers occurred at a higher frequency in those with SA. Our data suggest that SA is, in addition to lobular neoplasia, a predictor of multicentric breast cancers.
No preview · Article · Apr 2015 · Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin
[Show abstract][Hide abstract] ABSTRACT: To determine which immunohistochemical markers are useful for the identification of neoplastic myoepithelial cells in adenomyoepithelioma of the breast, the expression of seven myoepithelial markers (α-smooth muscle actin (α-SMA), calponin, p63, CD10, cytokeratin 5/6, cytokeratin 14, and S-100) was examined in 19 lesions from 16 patients. The lesion consisted of seven spindle and 12 clear cell lesions. For normal myoepithelial cells, α-SMA, calponin, and p63 were significantly more sensitive than cytokeratin 5/6, cytokeratin 14, and S-100. There was no significant difference in the expression of α-SMA, calponin, p63, and CD10 in neoplastic myoepithelial cells of adenomyoepithelioma regardless of spindle or clear cell types. In spindle cell lesions, high-molecular weight cytokeratins (HMWCK; cytokeratin 5/6 and cytokeratin 14) tended to show higher staining scores and S-100 showed lower staining scores than other markers. In clear cell lesions, HMWCK showed significantly lower staining scores than the other five markers. There was no significant difference in staining scores among the other five markers. HMWCK showed a unique paradoxical staining pattern in clear cell lesions, with diffusely positive inner epithelial cells and completely negative outer myoepithelial cells. Although the sensitivity of HMWCK in clear cell lesions is low, with this unique paradoxical staining pattern and relatively high sensitivity in spindle cell lesions, HMWCK could be useful in diagnosing adenomyoepithelioma. In choosing immunohistochemical markers, any of the seven markers are useful, but combining HMWCK and any one of α-SMA, calponin, and p63 would be a good panel for the diagnosis of adenomyoepithelioma.
No preview · Article · Dec 2014 · Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin
[Show abstract][Hide abstract] ABSTRACT: We report the first case of combined hepatoid and serous adenocarcinoma arising in the uterine corpus here. The patient is a 63-year-old Japanese woman. She presented with vaginal abnormal bleeding and subsequent imaging analysis disclosed a mass in the uterine corpus. This article is protected by copyright. All rights reserved.
[Show abstract][Hide abstract] ABSTRACT: The apocrine type of encapsulated papillary carcinoma (ECP-A), of the breast is a rare neoplasm and there are only eight cases reported to date. Herein, we report the ninth case. A 68-year-old Japanese woman presented with a left breast mass. The cytoplasm of the tumour cells showed positive immunostaining for GCDFP-15. Myoepithelial cells were absent within the papillary structures and at the periphery of the lesion. The clinical course of the patient was uneventful 11 months after surgery. We postulate that EPC-A belongs to the molecular apocrine type of breast carcinoma.
No preview · Article · Aug 2014 · The Malaysian journal of pathology
[Show abstract][Hide abstract] ABSTRACT: ABSTRACT Immunological status of patients with EBV-positive diffuse large B-cell lymphoma (EBV(+)DLBCL) without obvious immunodeficiency has not been elucidated. A multi-center prospective study was conducted to assess pretreatment T-cell responses to EBV, EBV-DNA load, and anti-EBV antibody in these patients. The proliferative and interferon (IFN)-γ-producing capacity of T-cells in response to autologous B-lymphoblastoid cell lines was determined using CFSE-based assay. Frequencies of EBV-specific CD4+ T-cells in EBV(+)DLBCL patients (n=13) were significantly higher than in healthy controls (HCs) (n=16) after both ex vivo and in vitro stimulation. Frequencies of EBV-specific CD8(+) T-cells in EBV(+)DLBCL patients tended to be higher than HCs after in vitro stimulation. EBV(+)DLBCL patients also showed increased IgG responses to lytic EBV-encoded antigens. Pretreatment plasma EBV-DNA level was significantly higher in EBV(+)DLBCL patients than EBV(-)DLBCL patients or HCs. In conclusion, EBV-specific T-cells showed increased reactivity, accompanied by higher levels of plasma virus DNA in patients with EBV(+)DLBCL.
No preview · Article · Jun 2014 · Leukemia and Lymphoma
[Show abstract][Hide abstract] ABSTRACT: A new imaging system based on an x-ray Talbot-Lau interferometry was developed. The preclinical study with mastectomy specimens was conducted, and the three types of images, i.e., the attenuation contrast(ATT) image, the differential phase contrast(DPC) image, and the x-ray small angle scattering(SAS) image, obtained by the system were compared to the pathological result. As a result, the SAS image showed micro-calcifications clearly. On the other hand, the inside of the mass with invasive carcinoma was visualized with relatively lower signal. The SAS image seemed to correspond to the homogeneity of the breast tissues. The breast images obtained with Talbot-Lau interferometry showed the different aspects which cannot be depicted with the conventional x-ray image. Comparative reading of the three images would enable us to get additional information of breast tissues.
[Show abstract][Hide abstract] ABSTRACT: Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant inherited disorder characterized by clinical features of skin lesions, pulmonary lesions and renal tumor. The gene responsible for this syndrome is located on chromosome 17p11.2 and designated as FLCN. In this article, we review renal tumors associated with BHDS with a focus on clinical and pathobiological aspects. Renal tumors often occur multifocally or bilaterally in the imaging analyses or gross examination. Histological examination of renal tumors includes a variety of subtypes such as hybrid oncocytic tumor, chromophobe renal cell carcinoma (RCC), oncocytoma, clear cell RCC and papillary RCC. The histologic discordance in multiple tumors seems to be characteristic of this syndrome. Oncocytosis is observed histologically in about half of the cases. Several investigations have elucidated that folliculin may be involved in the mammalian target of rapamycin (mTOR) pathway recently. Renal tumors composed of clear cells may behave in an aggressive fashion. However, renal tumors including hybrid oncocytic tumor, chromophobe RCC and oncocytoma behave mostly in an indolent fashion.
No preview · Article · Jun 2014 · Polish journal of pathology: official journal of the Polish Society of Pathologists
[Show abstract][Hide abstract] ABSTRACT: In this paper, we searched for the common histologic features characteristic of renal tumors in patients with Birt-Hogg-Dubé syndrome (BHDS). We selected six patients with histologically confirmed renal tumor in BHDS. Germline FLCN gene mutation has been identified in five patients. Multifocality and bilaterality of the renal tumors were pathologically or radiologically confirmed in five and two cases, respectively. Histological subtypes of the dominant tumor included three previously described hybrid oncocytic tumors, one composite chromophobe /papillary/clear cell RCC and one unclassified RCC resembling hybrid chromophobe/clear cell RCC. In one case, chromophobe RCC and clear cell RCC were separately observed. Small papillary lesions located in the peripheral area of the tumor, which we designated as intratumoral peripheral small papillary tuft (ITPSPT), were identified in all patients. In conclusion, multifocality/bilaterality of renal tumors, discordance of histologic subtypes and the presence of ITPSPT may be important clues to identify BHDS -associated renal tumors.
No preview · Article · Jun 2014 · Annals of diagnostic pathology
[Show abstract][Hide abstract] ABSTRACT: The introduction of combined antiretroviral therapy (ART) has reduced the mortality of patients with human immunodeficiency virus-1 infection worldwide. However, malignant lymphoma is a severe and frequent complication seen in patients with acquired immunodeficiency syndrome (AIDS). The diagnostic criteria for some categories of AIDS-related lymphoma were revised in the World Health Organization International Classification of Lymphoma, fourth edition. The purpose of this study was to assess the clinicopathological characteristics of Japanese patients with AIDS-related lymphoma according to the revised classification. In this retrospective study, 207 AIDS-related lymphoma cases diagnosed between 1987 and 2012 in Japan were subjected to histological subtyping and clinicopathological analyses. Diffuse large B-cell lymphoma (DLBCL) was the predominant histological subtype throughout the study period (n = 104, 50%). Among the DLBCL cases, 24% were of the germinal center (GC) type and 76% were of the non-GC type. Non-GC-type cases showed a significantly lower 1-year survival rate (43%) than the GC-type cases (82%). Cases of Burkitt lymphoma (n = 57, 28%), plasmablastic lymphoma (n = 16, 8%), primary effusion lymphoma (n = 9, 4%), Hodgkin lymphoma (n = 8, 4%), and large B-cell lymphoma arising in Kaposi sarcoma-associated herpesvirus-associated multicentric Castleman disease (n = 2, 1%) were also observed. Hodgkin lymphoma was more common in patients receiving ART (11.1%) than in ART-naïve patients (1.4%). Statistical analyses identified CD10 negativity, BCL-6 negativity, Epstein-Barr virus positivity, and Kaposi sarcoma-associated herpesvirus positivity as risk factors for poor prognosis. This information will help in the early diagnosis of lymphoma in patients with AIDS.
[Show abstract][Hide abstract] ABSTRACT: This study presents the assessment of trace elements in groundwater in Fuchu city located in western Tokyo, during 2012. Water samples were collected almost twice a month from two aquifers at depths of around 30-m (C1) and 45-m (C2) using a 50-m long well. Concentrations of 15 trace elements such as Li, B, Al, Cr, Mn, Fe, Ni, Cu, As, Se, Sr, Cd, Sb, Pb and Si, as well as pH, EC, Eh and DO were monitored. Standard multivariate analyses, such as Principle Component Analysis, were employed to characterize and to evaluate the variation of each element in groundwater. Although there is a 5-m impermeable silty layer between two aquifers, piezometric heads are about the same for both aquifers. There were significant differences in the concentrations of Li, B, Mn, Fe, As, Se, and Si in C1 and C2. These differences may be attributed mainly to difference in retention periods between two aquifers. The concentration of As varied from under detection limit to 8 μg/L. All trace element concentrations observed at the study site were under the environmental quality standard issue by Japanese Ministry of the Environment. The multivariate analysis indicates Si controls mainly trace element distributions and their mobility in groundwater.
[Show abstract][Hide abstract] ABSTRACT: Small intestinal GIST is relatively rare disease, and is difficult to diagnose in early stage for poor symptoms. A 51-year old man was transferred from other hospital due to abdominal pain and bloody discharge getting worse. On admission, abdominal CT scan showed a large tumor with gas and fluid lesion in the pelvis. We performed an emergent surgery, having diagnosed as perforating abscess. On intra-operative findings, there was a large tumor measured 12cm in the lower abdomen with abscess, and, that was growing on the antimesenteric side of the small intestine 50cm oral side of ileocecal valve. Partial resection of the intestine was performed, but we could not resect the tumor completely because of the invasion of surrounding organs and peritoneal dissemination. On immunohistochemistry, the tumor cells were positive for c-kit and CD34. The tumor was diagnosed as small intestinal GIST with liver metastasis and peritoneal dissemination. After surgery, imatinib therapy led to marked tumor shrinkage and almost complete clinical remission. Small intestinal GIST with perforating abscess is rare; a case with which concurrent metastasis present that had got more than 5-year survival has been seldom reported in Japan.
No preview · Article · Jan 2014 · Nihon Gekakei Rengo Gakkaishi (Journal of Japanese College of Surgeons)
[Show abstract][Hide abstract] ABSTRACT: NUT midline carcinoma (NMC) is recognized as a very rare tumor that most often occurs around the midline and shows NUT rearrangement. This tumor affects children and younger adults, progresses rapidly, and shows an extremely poor prognosis, even with intensive chemotherapy. Very few reports have described effective treatment for this tumor.
A 12-year-old girl with NMC was treated using cisplatin (CDDP), docetaxel, gemcitabine, pemetrexed, and vinorelbine.
Imaging showed partial response with CDDP and docetaxel, and complete response with gemcitabine. After reexacerbation of the tumor, although partial response was achieved with vinorelbine, the patient died 89 weeks after onset because of reexacerbation.
NMC is a very rare disease with poor prognosis. This study is the first to report response of NMC to gemcitabine and vinorelbine. The findings suggest that combination chemotherapies including CDDP, docetaxel, gemcitabine, and vinorelbine may be a choice in the treatment for NMC.
No preview · Article · Dec 2013 · Journal of Pediatric Hematology/Oncology