[Acute myocardial damage from a pheochromocytoma]. / Danno miocardico acuto da feocromocitoma.

Cardiac involvement in pheochromocytoma is rare but may be associated with serious clinical deterioration. A 70-year-old woman arrived at our Emergency Department because of chest discomfort, blood pressure lability, mild dyspnea and electrocardiographic signs suggesting an acute myocardial infarction. However two-dimensional echocardiogram did not show any segmental wall motion abnormalities but diffuse and severe left ventricular hypokinesia. The patient was treated with ACE-inhibitors and diuretics and did not receive thrombolytics or beta blocking agents. Creatine kinase-MB and troponin I were normal. Electrocardiogram and echocardiogram completely returned to normal within 1 week and a coronary angiography demonstrated normal coronary arteries. An increase in the catecholamine concentration in a 24-hour urinary sample suggested a pheochromocytoma that was confirmed by abdominal computerized tomography. During surgery, marked hypertension developed treated with sodium nitroprusside and labetalol, and after removal of the tumor severe hypotension required infusion of norepinephrine for several days.

[Benign hemangiopericytoma of the bladder: description of a case and review of the literature]. / Emangiopericitoma benigno della vescica: descrizione di un caso e revisione della letteratura.

Hemangiopericytoma (HPC) of the viscera is very rare and only six of these tumors arising in the urinary bladder have been previously reported. The differentiation of HPC from other neoplasms with prominent vascular pattern may be a diagnostic problem. The difficulty is further enhanced by the lack of distinctive immunohistochemical features of this tumor. Here is presented a case of benign HPC occurring in the right lateral wall of the bladder of a 37-year-old woman. The mass is completely transurethrally resected. Histologically, the tumor is characterized by a monomorphic population of polygonal or spindle-shaped cells packed around branching vascular channels, with "staghorn" configuration. There are no foci of necrosis or hemorrhage. The mitotic count is 2 per 10 HPF. Immunohistochemically, the tumor cells react with anti-bodies against Vimentin. Cytokeratin, Actin HHF35, S-100, CD 34, Factor VIII-related antigen are not expressed within the lesion. Ultrastructurally, the tumor cells surround endothelium-lined vascular channels. There are pinocytotic vesicles on the cell borders. The cells are separated from one another by deposits of basal lamina-like material and collagen. On the basis of clinical, histological, immunohistochemical and ultrastructural observations, we believe that the case reported is a primary benign HPC of the bladder. There is no evidence of recurrence or metastasis 32 months later.

Fibrillary and immunotactoid glomerulopathies.

There is sufficient clinical and morphological evidence to suggest that Fibrillary Glomerulonephritis (FGN) and Immunotactoid (IT) Glomerulopathy are two different diseases. Is still open to debate if IT glomerulopathy is a distinct entity or is strictly associated with a spectrum of systemic syndromes ("forme fruste" of Cryoglobulin and paraprotein associated diseases). Further studies about pathogenetic mechanisms of fibril or microtubule formation may allow a better understanding of these diseases.

Cytologic detection of metastatic malignant melanoma in urine. A report of three cases.

BACKGROUND: The presence of melanoma cells in urinary specimens is a rare event in either primary or metastatic melanoma of the genitourinary tract. CASES: Melanoma cells were observed in urinary specimens from three white males aged 50, 67 and 51 years, with a previous history of cutaneous melanoma in the first two cases; in the last one autopsy showed a primary melanoma of the gallbladder. The first patient, treated with palliative therapy, survived 10 months, the second died a few days after the cytologic diagnosis, while the third survived 1 month. All patients had widespread metastases at the time of cytologic diagnosis. CONCLUSION: The presence of melanoma cells in urinary specimens may be an important marker for assessing the spread of metastatic disease. When the cytologic diagnosis is made, widespread dissemination is present, and only palliative treatment is suggested.

Primary malignant mesothelioma of the tunica vaginalis of the testis. Immunohistochemistry and electron microscopy.

A case of primary malignant mesothelioma of the tunica vaginalis of the testis in a 75 year old man is here presented. Anamnesis for asbestos exposure was negative. Recurrent left hydrocele was the main symptom. Echography revealed a nodular mass in the caudal aspect of the epididymis and papillary projection on the surface of the tunica vaginalis of the testis. An inguinal left orchiectomy was performed. The tumor both in the solid area and in the papillary projections was identified as a malignant mesothelioma of epithelial type. The role of immunohistochemistry and ultrastructure for a correct definition of the tumor is underlined.

Rhabdomyosarcoma presenting as acute hematologic malignancy: case report and review of the literature.

The authors describe a case of undifferentiated rhabdomyosarcoma from unknown primary site, presenting as an acute hematologic malignancy with generalized lymphadenopathy, extensive bone marrow involvement and clinical and laboratory features of disseminated intravascular coagulation. Such a peculiar behaviour is known for rhabdomyosarcoma but is rare and can be a serious diagnostic problem for the clinician and the pathologist. The importance of a large spectrum immunohistochemistry as first diagnostic approach to any undifferentiated small-cell malignant tumor is stressed, together with the knowledge of the different immunoreactivity patterns. Desmin, MS-actin and myoglobin are the most reliable markers of this type of myogenic sarcoma.

Minimal change nephrotic syndrome with cecum adenocarcinoma.

Membranous glomerulonephritis is the most common glomerular disease associated with malignancy, the association of minimal change glomerulopathy with solid tumor is still uncommon. We report a 72-year-old man with nephrotic syndrome due to minimal change glomerular disease; an accurate seek of underlying malignancy revealed a cecum adenocarcinoma. We had a complete remission of nephrotic syndrome after surgery of carcinoma.

CT demonstration of liquid intratumoral fat layering in a necrotic renal cell carcinoma.

We describe a case of clear-cell adenocarcinoma of the kidney with CT evidence of fat that contradicts the rule that radiologically demonstrable fat is absent in renal carcinoma. The cyst-like appearance, egg-shell parietal calcifications, and extrarenal development of the mass suggested a preoperatively incorrect diagnosis of teratoma.

[Microcystic adenoma of the pancreas. Immunohistochemical and ultrastructural study of 7 cases]. / L'adenoma microcistico del pancreas. Studio immunoistochimico e ultrastrutturale di 7 casi.

Microcystic cystadenoma of pancreas is a rare benign neoplasm with peculiar pathologic characteristics. It is more frequent in women and in elderly age. Macroscopically it is composed of a spongy, microcystic mass with a starry central fibrous core. Histologically serous cysts are lined by cuboidal or flattened epithelium without nuclear atypias and with PAS positive cytoplasms. A surgical conservative approach is the treatment of choice. Seven cases of microcystic cystoadenomas of the pancreas observed within ten years at Osp. San Carlo Borromeo-Milano and Osp. Santa Maria degli Angeli-Pordenone from 6 women and 1 man aged 54-87 years are described. The preoperative and intraoperative investigations that have been necessary for reaching its diagnosis are discussed.

[Pancreatic endocrine F-cell tumor]. / Tumor pancreático endocrino de células F.

Two cases of pancreatic F-cell malignant tumors, producing only pancreatic polypeptide (PP) with clinical, biochemical and immunohistochemical features are described. No PP-associated endocrine symptoms were present. In both tumors, definitive diagnosis was immunohistochemical, and surgical treatment was carried out. Clinical evolution in the two patients was different: one of them died within the postoperative period with upper gastrointestinal bleeding by multiple duodenal ulcers, whereas the other--with lymph node metastasis at intraoperative diagnosis--is alive six years after treatment. F-cell tumors can be classified among the group of silent endocrine tumors of the pancreas. The reason for their discrepancy in the clinical behavior is unknown.

[Fibrous hamartoma in childhood]. / Amartoma fibroso dell'infanzia.

Is a rare soft tissue benign tumor usually appearing during the first two years of life, often occurring in axillary or upper arm region subcutaneous tissue. Surgery is the treatment of choice. The lesion histologically consists of three different tissue elements: dense mature fibrocollagenous, loose immature myxoid mesenchymal and mature adipose tissue. A case of fibrous hamartoma of infancy with the usual clinical characteristics, but with particular microscopic features due to the abundance in fibrocollagenous and scarcity of myxoid tissue, is described. The results of the immunohistochemical and ultrastructural study on the same patient are reported. The differential diagnostic problems are discussed.

[Microcystic serous cystadenoma of the pancreas. Enucleation or regulated pancreatic resection?]. / Cistoadenoma sieroso microcistico del pancreas. Enucleazione o resezione pancreatica regolata?

Cystic benign tumors of the pancreas are rare lesions: they represent less than 10% of all pancreatic neoplasms. We studied two cases of microcystic adenoma, one of the less common varieties, that only in the last few years has been morphologically recognized as a distinct pathological entity. Our clinical experience--though limited--made us remark some interesting features: 1) preoperative diagnostic procedures (ultrasonography and CT) may present great interpretative difficulties: in one of the cases observed, radiographic imaging demonstrated an unresectable pancreatic tumor. On the contrary, in both cases surgical radicality has been achieved; 2) preoperative needle aspiration cytology (FNAB) does not always provide diagnostic material, as happened in both case observed; 3) the diagnosis of a benign tumor may result from surgical exploration and multiple biopsies with intraoperative frozen sections. Further and more complex histological investigations will definitively diagnose a microcystic adenoma; 4) in disagreement with some authors' results, we observed that microcystic adenoma may grow rapidly: one of our patients, after an incomplete tumor enucleation, developed a local relapse in a few months, needing a subtotal distal pancreatectomy, followed by complete recovery; 5) surgical radicality is mandatory. This result can be achieved through a simple, but complete, tumor enucleation or through a pancreatic resection; 6) our immunohistochemical and ultrastructural studies on microcystic adenoma exhibited no cells with endocrine activity, supporting the hypothesis that this tumor may originate from ductal or centroacinar cells.

Glomerulonephritis induced by human IgMK-IgG cryoglobulins in mice.

BACKGROUND: Human cryoglobulinemia is sometimes associated with glomerulonephritis (GN) due to deposition of cryoglobulins (cryos). To see whether human cryos can induce GN in mice and to study time-related changes of glomerular lesions and possible factors of cryos' nephritogenicity, we developed an experimental passive model of cryoglobulinemic GN. EXPERIMENTAL DESIGN: Two cryos IgMk-IgG from 2 patients with active GN (OLD and SOR), 2 cryos IgMk-IgG (TAC and GRO) and 1 IgM lambda (CHI) from 3 patients without GN were purified, solubilized at 37 degrees C and injected intravenously into BALB/c mice, 4 mg, twice a day. To study the possible factors of cryo nephritogenicity, we analyzed: (a) the presence, amount, and size of complexed IgMk-IgG at 37 degrees C by fast flow liquid chromatography; (b) the Cc1 or Lc1 subclass of rheumatoid factors; (c) the isoelectric points of the IgMks; (d) The proportion of IgG subclasses in cryos. RESULTS: On day 1 from the beginning of intravenous injections, cryos OLD had induced mesangial deposits of human IgM, human IgG, mouse C3 and mesangial hypercellularity. On day 2, phagocytizing cells were found along with massive endoluminal and subendothelial deposits of IgM, IgG, and C3. On day 6, perivascular infiltrates of mononuclear cells were also seen. Cryos SOR induced a similar but milder form of GN. After administration of purified OLD IgMk, OLD IgG, GRO IgMk or GRO IgG, only OLD IgMk was deposited in the mesangium. Analysis of all the cryos revealed that: the amount of complexed IgMk-IgG at 37 degrees C was always less than 1% of cryos; Cc1 and Lc1 idiotypes were not related to the nephritogenicity of cryos, the isoelectric points of IgMks were 4.5 to 5.5 and IgG1 was the prevalent subclass. CONCLUSIONS: Data demonstrate that human cryos from patients with GN can induce GN in mice that resembles the corresponding human pathology. The affinity of IgMk for glomeruli and the unexpectedly small amounts of IgMk-IgG complexes at 37 degrees C suggest that there is a role of in situ binding in nephritogenicity which is independent of the isoelectric point, rheumatoid factor idiotype, or IgG subclass.

[Optical and ultrastructural morphology in hyperparathyroidism during dialysis]. / Morfologia ottica ed ultrastrutturale dell'iperparatiroidismo in corso di dialisi.

Twenty-six cases of surgically removed parathyroid glands in patients under chronic dialytic treatment were studied. In 19 cases a total parathyroidectomy was performed while in the remaining 7 cases only a subtotal parathyroidectomy was possible. All parathyroid glands (98) showed chief cells hyperplasia, with nodular pattern in 24 patients and diffuse pattern in two cases. A solid pattern was observed in 79 parathyroid glands, a trabecular in 49 and a follicular one in 33. Ultrastructural study was performed on 75 parathyroid glands. Electron microscopy has been useful in the evaluation of functional status of the single cell and in the detection of some morphological features (i.e., presence of intermediate filaments in the cytoplasms, mitochondrial abnormalities, multinucleation), rarely observed in hyperplasia and more frequent in adenoma. No morphologic differences have been found between hyperplastic glands and adenomatous glands. In the 27.2 per cent (out of 44 cases) flow-cytometric DNA analysis showed an aneuploid DNA content.

[The use of flow cytometry in the study of parathyroid hyperplasia and adenomas]. / L'impiego della citofluorimetria nello studio delle iperplasie e degli adenomi paratiroidei.

To discriminate benign from malignant parathyroid glands lesions is often difficult, because the morphologic features lack absolute diagnostic value. The differential diagnosis between chief cell hyperplasia and parathyroid adenoma is frequently based on physical features such as increased gland weight and number of enlarged glands. A carcinoma is identified by the evidence of local invasion, metastases or recurrence. Nevertheless the lesions classified as benign for lack of histologic features of aggressiveness can show nuclear atypia, increased mitotic figures and broad fibrous bands. Since DNA aneuploidy is present in a great number of human neoplasms and DNA aneuploidy has been suggest to be a marker of malignancy, flow cytometric assessment of ploidy appeared a possible method for rapid and objective distinction between benign and malignant lesions. Flow cytometric DNA content was evaluated on 113 parathyroid glands: the parathyroids were resected from 26 patients with hyperparathyroidism and from 17 patients with adenoma. The analysis was performed on paraffin-embedded specimens according to Hedley with minor modifications. Interpretable histograms were obtained for 103 parathyroids gland (mean CV = 5.3). Aneuploidy was detected in 22.5% of glands; in 12 instances of parathyroid hyperplasia the glands of the same patient showed different DNA Indexes. Cytometric results and morphological features do not correlate as far as aneuploidy and cellular atypia are involved. Although our results fail to show any correlation between morphology of parathyroid cells and DNA content, and abnormal DNA content suggests a careful follow up of these patients.

[Anatomopathologic pictures of chronic pancreatitis]. / Quadri anatomopatologici delle pancreatiti croniche.

Morphology of pancreas (either esocrine, either endocrine) was studied in 29 cases of surgically treated chronic pancreatitis (27 cases of chronic calcifying pancreatitis and 2 cases of chronic obstructive pancreatitis). Parenchymal sclerosis in chronic calcifying pancreatitis (CCP) which represents the goal of our study was graded as mild (10 cases), moderate (10) and severe (7). Immunoperoxidase staining (PAP method) for insulin, glucagon, somatostatin, pancreatic polipeptide (PP), vasoactive intestinal polipeptide (VIP) and gastrin, was used to investigate endocrine pancreas. Acinar sclerosis and endocrine damage were closely related. Progression of sclerosis into islet appears to follow vascular pedicles producing a fragmentation into small cell groups as final result. In all cases of moderate or severe sclerosis, A/B cell ratio was increased due to the reduction of insulin positive cells. "Adenoma-like complexes", i.e., apparent concentration of islets, resulting from the loss of the acinar component, were observed in 7 cases with moderate or severe sclerosis. Nesidioblastosis was a prominent feature in all cases but one, with a positivity for insulin in 11 cases, for glucagon in 13, for somatostatin in 6 and for PP in 17. No positivity for gastrin was observed, while VIP was detected in a few ganglia. An increased amount of PP cells in islet and budding from the ducts was noticed and their presence outside the pancreatic head was demonstrable in 4 out of the 7 distal pancreatectomy specimens. Our data confirm the secondary involvement of the endocrine pancreas in the sclerotic acinar process.

Ganglioneuroblastoma and urticaria by physical agents.

A case is reported of ganglioneuroblastoma in a 5-year-old boy, who had urticaria syndrome by physical agents (water, light, cold) in the last 2 years, associated with an apparent splenomegaly. A sonography and a CT scan showed a large cystic mass with calcification near the left kidney. After surgery, it was possible to diagnose left adrenal ganglioneuroblastoma (Evans's first stage). Complete regression of urticaria syndrome was obtained after mass removal.