[Giant cardiac metastasis of myxoid liposarcoma causing cardiac tamponade; report of a case].

We report a very rare case of cardiac metastasis of myxoid liposarcoma. A 55-year-old man presented with dyspnea. Two and a half years ago, he underwent resection of myxoid liposarcoma in the left thigh. Magnetic resonance imaging (MRI) revealed a giant tumor occupying the pericardiac cavity and pressing the heart and consequently causing cardiac tamponade. The patient underwent surgery through a left thoracotomy approach. The pericardiac cavity was filled with a giant tumor with a stalk from the right ventricle and 2 small nodules on the main pulmonary artery. He was relieved from the symptom: however, he had a recurrence of the tumor at the same site 5 months after the operation. He underwent surgery for the removal of the second tumor; however, he died 49 days after the operation. Although cardiac metastasis is a very rare condition, its awareness is essential for careful long-term follow-up for the early detection of a metastatic cardiac liposarcoma after the resection of the primary tumor.

Secretory carcinoma of the breast: a tumour analogous to salivary gland acinic cell carcinoma?

AIMS: Acinic cell-like breast carcinoma is a newly recognized entity, and few acinic cell-like breast carcinoma cases have been reported. All reported acinic cell-like breast carcinomas were counterparts of the solid type of acinic cell carcinoma of the salivary gland. We report here three cases of secretory breast carcinoma with acinic cell differentiation, and discuss the similarity between secretory breast carcinoma and acinic cell carcinoma of the salivary gland. METHODS AND RESULTS: The cases were histologically identical to acinic cell carcinoma of the salivary gland: papillary-cystic type in case 1, a mixture of papillary-cystic, microcystic and follicular type in case 2, and microfollicular type in case 3. Immunohistochemically, the tumour cells were positive for salivary-type amylase, lysozyme, S100 protein and alpha 1-antitrypsin, and negative or less reactive for gross cystic disease fluid protein-15 and oestrogen receptor. All three cases did not reveal metastasis or recurrence. CONCLUSIONS: These cases were typical of secretory breast carcinoma, and were clinically, histologically and immunohistochemically analogous to acinic cell carcinoma of the salivary gland. We emphasize that secretory breast carcinoma and acinic cell carcinoma of the salivary gland may be identical lesions.

International neuroblastoma pathology classification for prognostic evaluation of patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group.

BACKGROUND: The International Neuroblastoma Pathology Classification was established in 1999 for the prognostic evaluation of patients with neuroblastic tumors (NTs). METHODS: Pathology slides from 746 NTs (the Children's Cancer Group [CCG]-3881 and CCG-3891 studies) were evaluated according to the International Classification. First, prognostic effects of the morphologic indicators (grade of neuroblastic differentiation: undifferentiated [U], poorly differentiated [PD] and differentiating [D]; and mitosis-karyorrhexis index [MKI]: low [L-MKI], intermediate [I-MKI], and high [H-MKI]) for tumors in the neuroblastoma (NB) category were tested. Then, prognostic significance of the International Classification for all NTs in four categories (neuroblastoma [NB]; ganglioneuroblastoma, intermixed [GNBi]; ganglioneuroma [GN]; and ganglioneuroblastoma, nodular [GNBn]) was analyzed. Finally, age distribution of the patients in the four categories as well as three subtypes (based on the grade of differentiation) in the NB category was compared. RESULTS: There were 630 NB tumors, 30 GNBi tumors, 10 GN tumors, and 76 GNBn tumors. In the NB category, prognostic effects of the indicators (three grades of differentiation and three mitosis-karyorrhexis index [MKI] classes: low [L], intermediate [I], and high [H]) were affected significantly by the age of the patients. The age-linked evaluation of the indicators according to the International Classification successfully distinguished two prognostic subgroups: the favorable histology (FH) subgroup (PD/D and L/I-MKI tumors in patients age < 1.5 years, D and L-MKI tumors in patients ages 1.5-5.0 years; 90.4% 5-year event free survival [EFS]) and the unfavorable histology (UH) subgroup (U and/or H-MKI tumors in patients of any age, PD and/or I-MKI tumors in patients ages 1.5-5.0 years, any grade of differentiation, and any MKI class in patients age > or = 5 years; 26.9% EFS) (P < 0.0001). The International Classification also distinguished the FH group (FH subgroup with NB, GNBi, and GN tumors) and the UH group (UH subgroup with NB and GNBn tumors) for all NTs (90.8% EFS and 31.2% EFS, respectively; P < 0.0001) and provided independent prognostic information on both patient age and disease stage (P < 0.0001). Among patients with FH tumors, the median ages of patients with the PD and D subtype tumors in the NB category were 0.43 years (range, 0-1.50 years) and 1.50 years (range, 0.02-4.65 years), respectively, and the median ages of patients with GNBi and GN tumors were 3.51 years (range, 0.96-14.85 years) and 4.80 years (range, 1.94-17.05 years), respectively. In contrast, patients with UH tumors generally were older when they were diagnosed, and with median ages of 2.99 years (range, 1.30-8.84 years) for patients with U subtype tumors, 2.59 years (range, 0.0-12.57 years) for patients with PD subtype tumors, 2.16 years (range, 0.35-9.90) for patients with D subtype tumors, and 3.26 years (range, 0.57-15.90 years) for patients with GNBn tumors. CONCLUSIONS: This study confirmed the prognostic significance of the International Classification, substantiated age-linked prognostic effects of the morphologic indicators for patients with the tumors in the NB category, and supported the concept of an age-appropriate framework of maturation for patients with the tumors in the FH group.

Embryonal sarcoma of adult and pediatric kidneys: report of a case with localized submucosal invasion of the renal pelvis and long-term survival.

A case of cystic embryonal sarcoma of the kidney (CESK) with a rapidly fatal outcome was recently reported.1 Here, we report another case of a 12-year-old boy with a localized but ill-defined submucosal lesion of CESK in the right renal pelvis. The tumor consisted principally of small mesenchymal cells with oval to spindle nuclei and scanty cytoplasm, infiltrating in dense arrangements. Two growth patterns were distinguished in the tumor cells: (i) a diffuse infiltrating pattern without an epithelial component; and (ii) a foliated pattern with an epithelial lining over the surface. Foci of the diffuse pattern predominated over those that were lobular, infiltrating superficial layers of renal sinuses and along pyramids, in both of which remarkable intravenous invasion was evident. Foci of the foliated pattern invaded deeper portions of a few sinuses and frequently penetrated into their veins, producing together with their epithelial lining a characteristic foliated structure. Lining epithelial cells around lobular foci often appeared hob-nailed or eosinophilic in the cytoplasm. Despite the remarkable intravenous encroachment, the patient has remained well without a recurrence for more than 26 years after a simple nephrectomy. The present case report expands our understanding of the biological nature of CESK.

Gastrointestinal stromal tumor with skeinoid fibers of the ileum.

Gastrointestinal stromal tumor (GIST) is not uncommon among gastrointestinal nonepithelial tumors, but there are few reports describing the cytologic findings. We report a case of GIST with skeinoid fibers in scrape cytology preparation. The patient was a 53-year-old man with a tumor in the small intestine. Scrape preparations from the cut surface of the resected tumor revealed cellular material composed of spindle cells showing loose clusters or single cells. The nuclei were spindled, elongated or cigar-shaped, and relatively uniform. The cytoplasm was fragile and demonstrated a finely fibrillar material. Dense hyaline materials with irregular outline were observed within the loose clusters composed of the tumor cells. The hyaline materials were also observed in the background. Histologic preparation showed spindle cells arranged in a fascicular or storiform pattern. Most eosinophilic globules were distributed between the tumor cells. The globules were positive in periodic acid-Schiff reaction, and were stained blue with Masson's trichrome stain. Immunohistochemically, the tumor cells were strongly and diffusely positive for c-kit, focally and weakly positive for alpha-smooth muscle actin, and negative for CD34 and S-100 protein. We emphasize that skeinoid fibers are characteristic of GIST arising in the small intestine, and their presence predicts a good prognosis, even in malignant GIST.

Inflammatory pseudotumor of the spleen: a case report.

We report on an inflammatory pseudotumor of the spleen. A 72-year-old woman visited our hospital complaining of nausea. Physical examination and laboratory investigations were unremarkable. Ultrasonography, computed tomography, magnetic resonance imaging and angiography showed a hypovascular splenic mass measuring about 5 cm in diameter with a calcification in the center of the lesion. Splenectomy was performed. The removed spleen, weighing 145 g, contained a tan-white, circumscribed mass, measuring 6.2 x 5.5 x 5.3 cm. Histologically, the splenic mass was composed of an admixture of inflammatory cellular elements, predominantly plasma cells and lymphocytes with hyalinization, fibrosis, lymph follicles and multinuclear giant cells, suggestive of a inflammatory pseudotumor. The patient is currently alive and asymptomatic, 24 months after surgery. Inflammatory pseudotumors of the spleen are extremely rare and only 39 cases have been reported in the medical literature.

Solid-pseudopapillary carcinoma of the pancreas.

A case of solid-pseudopapillary carcinoma (SPC) of the pancreas in a 34-year-old Japanese woman is presented. An abdominal ultrasonography revealed a mass, which measured 10 cm in diameter, in the body and tail of the pancreas. The tumor was resected and it was originally diagnosed as a non-functioning islet cell tumor. One year and five months later, the patient was re-admitted to hospital, and liver metastasis was confirmed by ultrasonography. The patient died 6 days after the second transcatheter arterial embolization (TAE) required for the metastasis. The autopsy showed small foci of liver metastasis. A retrospective examination of the tumor suggested the diagnosis of SPC because of its characteristic solid and pseudopapillary structures, immunohistochemical findings, and liver metastasis. This case suggests that capsular invasion, specifically found at the surgical margin of the peritoneal side, may be an important pathological finding that is suggestive of malignant potential in solid-pseudopapillary tumor. If there is such a finding in a surgical specimen, an intensive follow up should be advised to the clinician.

Ectopic hamartomatous thymoma. Report of a case with fine needle aspiration biopsy findings.

BACKGROUND: Ectopic hamartomatous thymoma is a rare, benign tumor occurring exclusively in the supraclavicular and suprasternal regions. To the best of our knowledge, there are no English-language reports on its cytologic findings. CASE: A fine needle aspiration specimen from a mass in the suprasternal region in a 63-year-old male revealed epithelial cell nests, spindle cells, a cluster of mature adipocytes and a small number of lymphocytes. CONCLUSION: Although ectopic hamartomatous thymoma is very rare, fine needle aspiration cytology may contribute to the correct diagnosis in conjunction with the characteristic clinical findings.

Papillary squamous cell carcinoma of the uterine cervix: diagnostic pitfalls.

A case of papillary squamous cell carcinoma (PSCC) of the uterine cervix is reported. The patient was a 73-year-old Japanese woman with acute renal failure and bilateral hydronephrosis. A cauliflower-like mass was found in the uterine cervix. A uterine cervical biopsy specimen revealed PSCC in situ, while clinically it was an invasive carcinoma. Uterine cervical biopsy was performed a second time to confirm its stromal invasion. However, only small fragments were obtained because of heavy bleeding from the tumor and they showed PSCC in situ again. Following this, computed tomography of the pelvis revealed a 5 cm mass in the uterine cervix, invading the vagina and urinary bladder. Though deep-wedge biopsy, loop electrosurgical excision, or cone biopsy is recommended to evaluate PSCC, it may be impossible to perform any of these procedures because of bleeding such as that seen in our case. In these circumstances, good communication between pathologists and clinicians is important since lack of communication may cause PSCC to be microscopically misinterpreted as in situ carcinoma rather than invasive carcinoma.

Sclerosing lipogranuloma of the scrotum: report of a case with fine needle aspiration biopsy findings.

BACKGROUND: Sclerosing lipogranuloma of the scrotum is a rare granulomatous lesion of the subcutis. To the best of our knowledge, there have been no English-language reports dealing with its cytologic findings. CASE: A fine needle aspiration specimen from a mass in the scrotum in a 62-year-old male showed epithelioid cells, multinucleated giant cells and fibroblasts, which were suggestive of sclerosing lipogranuloma. The diagnosis was confirmed by histology of the resected specimen. CONCLUSION: Fine needle aspiration cytology is useful for the diagnosis of sclerosing lipogranuloma. It is important, however, to interpret the fine needle aspiration cytology specimen in conjunction with the clinical information.

Pathology review of screening negative neuroblastomas: a report from the Quebec Neuroblastoma Screening Project.

BACKGROUND: The Quebec Neuroblastoma Screening (QNS) Project completed a 5-year program for measuring urinary vanillylmandelic acid (VMA)/homovanillic acid (HVA) levels at age 3 weeks and/or 6 months in 89% of 476,603 Quebec-born infants from 1989-1994; 45 screening positive preclinical cases (S-positive cases) and 20 congenital/neonatal (C/N) cases were identified. As of April 1997, an additional 59 cases in the same birth cohort were diagnosed clinically; these neuroblastomas developed after screening verified normal VMA/HVA levels (S-negative cases). METHODS: Pathology specimens from 45 of 59 S-negative cases were reviewed centrally and classified according to the Shimada system. Results were compared with clinical data and also with S-positive and C/N cases. RESULTS: Of 45 S-negative cases, 27 tumors had favorable histology (FH) and 18 had unfavorable histology (UH). Approximately 52% of FH tumors were diagnosed before age 1 year, whereas UH tumors were nearly exclusively (94%) diagnosed after age 1 year (P < 0.01). Approximately 89% of FH tumors were Stage I, II, or IV-S, whereas 72% UH tumors were Stage III or IV (P < 0.001). All children with FH tumors were alive at last follow-up (range of follow-up period: 9-79 months; median, 35 months), whereas 8 children with UH tumors died of disease even after limited follow-up (range of follow-up period: 0-60 months; median, 20 months). By contrast, S-positive and C/N cases were predominantly (97%) FH tumors, often (76%) Stage I, II, or IV-S, with excellent clinical outcome (survival rate of 98%). CONCLUSIONS: The majority of the UH neuroblastomas that developed in the birth cohort of the QNS Project were included in the group of S-negative cases and could not be detected by the screening at age 3 weeks and/or 6 months.

Intraepithelial haemorrhage of the oesophagus: a terminal event in haematological disorders.

AIMS: To investigate the clinicopathological findings in cases with intraepithelial haemorrhage of the oesophagus (IHO). METHODS: Necropsy records and the histopathology findings in the oesophagus were reviewed for the period 1990 to 1995. Six cases (0.7%) of IHO were found among 919 necropsy cases. Clinical records of these patients and gross and microscopic slides were reviewed in detail. RESULTS: The ages of the IHO cases ranged from 42 to 82 years (average 68 years), with a male to female ratio of 1:2. All cases had underlying haematological disorders with thrombocytopenia, but disseminated intravascular coagulation was not evident in any case. Macroscopically, solitary (two cases) or multiple (four cases) haemorrhagic lesions ranging from 6 to 79 mm in size were identified within the distal oesophagus. Microscopically, there was no inflammatory infiltration, destruction of red blood cells, or submucosal scar formation. CONCLUSIONS: IHO seems to occur shortly before death as a terminal event in haematological disorders. Based on these observations, the term "terminal IHO" can be suggested for this type of oesophageal lesion.

Beta 2 microglobulin haemodialysis related amyloidosis: distinctive gross features of gastrointestinal involvement.

Two cases of beta 2 microglobulin amyloidosis following long term haemodialysis found during necropsy are reported. The patients were 59 and 65 year old Japanese men, respectively. In both cases, systemic distribution of beta 2 microglobulin amyloid deposits was observed. The gastrointestinal tract including the stomach, small intestine, and colon showed the distinctive gross feature of rippled appearance, which was characterised by serosal wrinkles along the muscle layer arrangement. These areas were confirmed to contain deposits of beta 2 microglobulin in the muscularis propria. Although the outline of the muscle layers was preserved, most muscle fibres, encircled by the amyloid deposits, were atrophic or had disappeared microscopically. In neither case could a definite diagnosis of amyloidosis be made while the patient was alive. Interestingly, the oesophagus presented less involvement compared to the remainder of the gastrointestinal tract. In comparison with the AA or AL type of amyloidosis, beta 2 microglobulin haemodialysis related amyloidosis showed a rippled appearance of the serosal rather than mucosal changes, which may explain the difficulty in diagnosing amyloid deposits using biopsies of the gastrointestinal tract.

Morphological changes and proliferative activity of alveolar epithelium in mouse lungs treated with urethan.

We have observed sequential cellular changes in type II pneumocytes in mouse lungs following oral administration of 0.1% urethan solution. Histologically, scatterings of swollen alveolar cells were first noticed 25 days after administration. These cells continued to swell further, after which they aggregated more compactly to form a papillary structure by day 75. After day 100, distinct tumour nodules were found with atypical large cells in some areas. Necrotic foci appeared in large tumours of more than 4 mm diameter. At day 250, nucleoli became distinct and large bizarre and multinucleated cells were intermingled with some mitotic figures. These morphologies correspond to conventional descriptions; that is, hyperplasia (day 25-75), benign neoplastic changes (day 75-100) and malignant neoplastic changes (day 100-250). The proliferating cell nuclear antigen (PCNA) and nucleolar organizer regions (AgNOR) scores in these lesions increased with time, and proliferative activities during tumour progression seemed continuous. However, proliferative activity of the cells on specific days did not differ statistically from the values on neighboring days. We speculate that the hyperplasia-like lesions seen in our mice are neoplastic in nature from their outset.

Very late activation antigen 4-vascular cell adhesion molecule 1 interaction is involved in the formation of erythroblastic islands.

Erythroblastic islands are anatomical units consisting of a central macrophage surrounded by erythroblasts. We studied the adhesion molecules involved in the formation of these structures. Central macrophages of erythroblastic islands isolated from the spleens of phlebotomized mice were clearly stained for vascular cell adhesion molecule 1 (VCAM-1). The surrounding erythroblasts of the erythroblastic islands strongly expressed the alpha 4 integrin of very late activation antigen 4 (VLA-4: alpha 4 beta 1 integrin), the counter receptor of VCAM-1, whereas most reticulocytes and erythrocytes did not. Both monoclonal antibodies (mAbs) against alpha 4 integrin and VCAM-1 disrupted the erythroblastic islands cultured in the presence of erythropoietin. Moreover, adhesion of splenic erythroblasts to tumor necrosis factor alpha-stimulated mouse splenic endothelial cells, which showed high expression of VCAM-1 but not intercellular adhesion molecule 1, was inhibited by the anti-VCAM-1 and anti-alpha 4 mAbs. These findings suggest that VLA-4-VCAM-1 interaction plays a crucial role in the formation of erythroblastic islands.

Malignant localized fibrous tumor of the peritoneum arising in its benign counterpart.

We report a case of malignant localized fibrous tumor (LFT) of the peritoneum in a 78-year-old man. The tumor was pedunculated and the malignant area completely surrounded by its benign counterpart, forming "a nodule within a nodule" appearance. Histologically, the latter area showed typical features of a benign LFT, while the former fulfilled the criteria of a malignant LFT as defined by England et al.; i.e., one being rich in fibroblastic spindle cells with plump and atypical nuclei, hyalinized stroma and mitotic figures. Immunohistochemically, spindle cells in both portions of the tumor were positively stained for vimentin but completely negative for keratin (AE1/AE3), S-100 protein and desmin. There was a significant increase in proliferating cell nuclear antigen (PCNA)-positive cells in the malignant portion (80%) in comparison with the benign portion (45%). To date, we are not aware of any report on a malignant localized fibrous tumor in association with a benign counterpart. The unique association, therefore, prompted us to report our experience, and suggests the possibility of malignancy developing in the benign localized fibrous tumor.

Malignant sex-cord stromal tumor of the testis: report of a case with special reference to its unusual intracytoplasmic substructures.

A case of malignant sex-cord stromal tumor (gonadal stromal tumor) in the left testis of a 42-year-old man with no sign of hormonal abnormality is reported. Histologically, a large portion of the tumor was composed of solid nests of round cells with a moderate amount of eosinophilic cytoplasm, but some areas contained small spaces somewhat resembling Call-Exner bodies and/or the follicular structures of granulosa cell tumors. In general, however, the tumor cells did not show a clear-cut differentiation into either Sertoli cells or granulosa cells. The tumor, because of its high mitotic rate, lymphatic, venous and capsular invasion, and metastasis to the para-aortic lymph nodes, was considered to be malignant. We simply designated this tumor, therefore, as a malignant sex-cord stromal tumor. An ultrastructural study revealed unique intracytoplasmic bodies composed of multiple stacks of tubular structures which superficially mimicked annulate lamellae. No lipid droplets, Reinke's crystalloids or Charcot-Böttcher filaments were seen. The rarity of the tumor and the presence of unusual substructures prompted us to report the case.

[A case of intrapulmonary schwannoma that should be distinguished from lung cancer].

A 32-year-old woman was referred to our division because of abnormal shadow on a chest X-ray film taken at an annual health survey. Chest X-ray films and chest CT scanning revealed a smooth-surface mass at the left hilus. A bronchoscopic examination revealed severe extraluminal compression in the left basal bronchus but no visible tumors. Surgical treatment was performed on the suspicion of lung cancer. Since the intraoperative frozen section examination revealed a benign tumor, the tumor mass was resected. Based on intraoperative and pathological findings, benign intrapulmonary schwannoma associated with the left basal bronchus was diagnosed. Only 15 cases of intrapulmonary schwannoma have been reported in Japan.