[Myeloid sarcoma in uterine leiomyoma]. / Sarcoma mieloide in leiomioma del miometrio.

In this case report we present a 44-year-old woman with a 2-weekhistory of vaginal bleeding. Gynaecological examination revealed the presence of a polypoid neoformation in the endometrial cavity with a maximum diameter of 4 cm. Histological analysis showed a classic leiomyoma infiltrated by a second monomorphic, highly undifferentiated neoplasia. Immunohistochemical analysis revealed a negative reaction for cytokeratin, CD10, inhibin, CD99, CD20, CD3, TdT and CD34, and positivity for CD45, MPO, CD68 and CD117. A diagnosis of myeloid sarcoma in myometrial leiomyoma was made. The following days the patient showed the onset of an acute myeloid leukaemia M5a. Forty days after diagnosis the patient died for complications related to immunodeficiency caused by therapy. Especially when a common antibody panel reveals negativity for epithelial, mesenchymal and lymphoid markers, this case underlines the importance of considering myeloid sarcoma in differential diagnosis of undifferentiated tumours arising in an extramedullary site in order to avoid errors and permit optimal therapeutic management.

Skin adnexal neoplasm closely resembling adenomatoid tumor: a unique occurrence.

We describe a primary skin neoplasm located in the left chest wall that closely resembled adenomatoid tumor of male and female genital tract. It occurred in a 52-year-old woman who had undergone a left quadrantectomy with regional lymphadenectomy for invasive ductal carcinoma of the breast 7 years previously. The tumor involved the dermis and subcutaneous tissue and measured 0.7 cm in greatest diameter. Immunohistochemical evaluation showed strong positivity for cytokeratin pool and negativity for CD31, calretinin, WT-180, and for estrogen and progesterone receptors. We are unaware of a previous description of this morphologic pattern in a primary skin tumor, which we have interpreted as of skin adnexal and specifically of eccrine sweat gland origin.

[Ultrasonographic, serologic, and clinical characteristics of a case of prostatic malacoplakia]. / Caratteristiche ecografiche, sieriche e cliniche di un caso di malacoplachia prostatica.

An additional case of malakoplakia of the prostate in described to highlight its clinical, echographic and serum features. It's a rarest disease especially when prostate is involved, but well known in world literature. This case is reported to describe more accurately its echographic patterns and also for growing up precision in linguistical expressions used by echographists. We hope to contribute in collecting data about an uncommon pathology. At last we discuss about differential diagnosis when biopsy is negative for neoplasia and how it's possible to end further biopsies.

Salivary duct carcinoma of the parotid gland: a case report.

The authors report a case of a salivary duct carcinoma (SDC) of the parotid gland revealed by a facial palsy. The clinical and pathological features of this highly malignant neoplasm are described. The aggressive nature of this form demands a combined chemo-radio-surgical treatment; only early recognition and treatment are likely to result in a favourable outcome.

Splenic lymphoma: unusual case with exclusive red pulp involvement.

A case of Primary Malignant Lymphoma of the Spleen (PMLS) with exclusive red pulp involvement is described and discussed. Although the unusual topographic presentation the authors emphasize the physiologic arrangement of lymphoid cell in splenic red pulp cords that can give origin to the neoplasia. They moreover discuss problems of differential diagnosis with Malignant Histiocytosis (MH), Hairy Cell Leukemia (HCL) and Myeloid Process, both by morphology and immunohistochemistry.

Hairy cell leukemia without splenomegaly nor myelofibrosis in a patient with gastric adenocarcinoma: early phase of the disease or a variant?

The first case of patient affected both by gastric carcinoma and hairy cell leukemia (HCL) is reported. From a clinical standpoint, this 54-year old man presented with striking leukopenia without splenomegaly. From a morphological point of view, the infiltration by leukemic cells occurred in the hypoplasic areas of the bone marrow biopsy (BMB) without increase in reticulin fibers. From these observations the authors deduce that: 1) BMB in many cases is the only morphologic tool for diagnosis and prognosis of HCL; 2) probably the "hypoplasic" variant of HCL will become more frequent, because of the increasing indication to BMB in course of pancytopenia, even in absence of splenomegaly; 3) our case is probably related more to an early phase of the disease than to a distinct variant. In addition, we propose that the co-existence of an aggressive solid tumor and HCL could be related to the immunosuppressive action by hairy cells.

[Monoclonal gammopathies: differential diagnosis with bone marrow biopsy]. / Le gammopatie monoclonali: diagnostica differenziale su biopsia osteomidollare.

Aim of the present study is to examine the morphological aspects of monoclonal gammopathies (GM) detectable by bone marrow biopsy. With reference to Multiple Myeloma (MM), the more important disease of this group from a clinical standpoint, other conditions will be later evaluated. Particular attention will be given to Monoclonal Gammopathy of Undetermined Significance (MGUS) that still today is characterized by wide shadows concerning both ethiology and evolution significance.

Lambert-Eaton myasthenic syndrome and polyneuropathy in a patient with epidermoid carcinoma of the lung.

We describe a patient affected by the Lambert-Eaton myasthenic syndrome, sensory motor axonal neuropathy and epidermoid carcinoma of the lung. Serum autoantibodies to voltage-operated calcium channels were detected. After lobectomy, voltage-operated calcium channel-related structures were demonstrated in the patient's tumor. By immunocytochemistry, the patient's IgG reacted with neural structures and particularly with intermediate filaments. We think that these autoantibodies may be implicated in the pathogenesis of the neurological symptomatology.

Chronic bilateral dacryo-adenitis in identical twins: a possible incomplete form of Sjögren syndrome.

We report an unusual case of chronic bilateral dacryo-adenitis in 10-year-old identical twin sisters. Both girls presented with bilateral lacrimal gland enlargement and developed moderate xerophthalmia and keratitis. Both the lacrimal and minor salivary gland biopsies showed a non-granulomatous inflammatory infiltration of mono-nuclear cells. All granulomatous diseases and neoplasms could therefore be ruled out and only Sjögren syndrome and very few other forms of chronic dacryo-adenitis remained as possible diagnoses. Both patients and their parents were evaluated for auto-antibodies. Very low titres of smooth muscle antibodies were found in one, antinuclear antibodies in two and anti-dsDNA antibodies in all four members of the family. Even though the titres of antinuclear and anti-dsDNA antibodies increased in one of the sisters, both patients did not develop any sign or symptom of a systemic connective tissue disease. During the 6 years' follow up, both patients showed persistent tarsal gland enlargement but no other symptoms apart from a moderate xerophthalmia and occasional mild keratitis.

Pancreatic cancer versus chronic pancreatitis: diagnosis with CA 19-9 assessment, US, CT, and CT-guided fine-needle biopsy.

The authors prospectively performed serum CA 19-9 assessment, ultrasound (US), computed tomography (CT), and CT-guided fine-needle aspiration biopsy (FNAB) of the pancreas in 81 consecutive patients with suspected chronic pancreatitis or pancreatic neoplasm. The final diagnosis was pancreatic cancer in 54 patients and chronic pancreatitis in 27 patients. CA 19-9 assessment, US, CT, and FNAB were considered nondiagnostic, respectively, in 0%, 25%, 19%, and 6% of cases. When a definite diagnosis was rendered, the positive predictive value was 90% for CA 19-9 assessment, 95% for US, 98% for CT, and 100% for FNAB; the negative predictive value was, respectively, 69%, 95%, 86%, and 100%. The accuracy of all diagnostic and nondiagnostic studies was 81% for CA 19-9 assessment, 72% for US, 77% for CT, and 94% for FNAB. It is concluded that CT-guided pancreatic FNAB is the most reliable examination for enabling differential diagnosis of pancreatic cancer and chronic pancreatitis. When the pancreas is well visualized at US, the negative predictive value for pancreatic cancer is more accurate than that of CA 19-9 assessment and CT.

[Fine needle aspiration of pancreatic masses. A study of 81 cases]. / Agoaspirato con ago sottile (FNAB) delle masse pancreatiche. Studio di 81 casi.

Study of 81 cases. Fine Needle Aspiration Biopsy (FNAB) was performed under computerized thomography scan (CT) in 81 patients with documented pancreatic mass (54 with neoplasia and 27 with chronic pancreatitis). This procedure, when associated to the immediate cytological evaluation of the adequacy of the smears, permits to obtain 100% in sensibility, specificity and predictive value of positive cases. These results and the study of the literature confirm CT-guided FNAB of the pancreas as the procedure of choice to resolve problems of differential diagnosis in pancreatic masses.

Diagnosis of malignant change in duodenal villous adenoma.

Villous adenomas of the duodenum are rare, and malignancy is discovered in about 30% of the lesions. The authors describe two cases of villous adenoma of the second portion of the duodenum (13 and 8 cm in diameter). The diagnosis was obtained through endoscopy, which did not demonstrate any malignant change. In both patients, malignant change was shown by intraoperative frozen sections, and a pancreatico-duodenectomy was performed. Review of the literature and the authors' experience indicate endoscopic biopsies do not rule out the presence of malignancy in adenomatous pathology of the duodenum. Therefore, laparotomy should be performed whenever endoscopic excision of the neoplasm is not feasible. The authors emphasize the importance of an intraoperative diagnosis based on accurate frozen sections and propose a correct procedure to obtain the best results.

LAK1: a novel leucocyte differentiation antigen shared by lymphoid and endothelial cells.

Lymphokine activated killer (LAK) cells have been utilized as a useful tool in cancer adoptive immunotherapy. The lineage origin of this population has always been controversial since it shares phenotypic markers with both myelomonocytic cells and T lymphocytes. Recently we described a new monoclonal antibody (MoAb), termed LAK1, which recognizes a 120 Kd surface molecule expressed on human large granular lymphocytes (LGL) and LAK precursors and effectors. LAK1 MoAb defines two different populations of positive cells amongst peripheral lymphocytes: the first subset (20%), represented by brightly stained cells, belongs to the non T-LGL population, whereas the second subset (30%) displays low fluorescence intensity and was partially composed of T lymphocytes. More interestingly, LAK1 is shared by some other cell types, such as monocytes and vascular endothelial cells. Immunohistochemical staining performed on muscle, endometrium and lymphoid or other non lymphoid tissues shows that LAK1 antigen is selectively expressed by the reticuloendothelial system.

CT-guided pancreatic percutaneous fine-needle biopsy in differential diagnosis between pancreatic cancer and chronic pancreatitis.

Differential diagnosis between pancreatic cancer and chronic pancreatitis is still difficult to establish. In 63 patients with suspected pancreatic neoplasm we performed: serum CA 19-9 assessment, abdominal ultrasound. CT scan and CT-guided pancreatic percutaneous fine-needle biopsy. The conclusive diagnosis was pancreatic cancer in 40 patients and chronic pancreatitis in 23 patients. With regard to the differential diagnosis, sensitivity and specificity were respectively 80% and 78% for serum CA 19-9, 75% and 65% for abdominal US. 85% and 70% for CT scan. 00% and 87% for percutaneous fine-needle biopsy. We conclude that CT-guided percutaneous fine-needle biopsy is the most reliable method for differential diagnosis between pancreatic cancer and chronic pancreatitis.

Langerhans histiocytosis of the choledochus in an adult patient with a history of disseminated disease.

A 43 year-old man with a 15-year history of disseminated Langerhans histiocytosis (LH) in complete clinical remission was admitted with jaundice and abdominal pain. Pathological examination demonstrated LH in the choledochus associated with sclerosing cholangitis in the liver. Immunohistochemistry for S-100 protein and electron microscopy of the choledochus tissue showed Langerhans cell-like elements in the infiltrate. Our findings suggest that, in patients with disseminated Langerhans histiocytosis, liver function should be monitored, particularly, for signs of biliary obstruction, and demonstrate that such a finding is possible in adults.

An uncommon cause of massive intraperitoneal bleeding: leiomyoblastoma of the stomach.

A large exogastric leiomyoblastoma in a 48-year-old male revealed by asymptomatic upper abdominal mass is reported. Abdominal ultrasound, computerized tomography scan and magnetic resonance showed a 20 cm cystic lesion apart from liver and pancreas of undetermined origin. During hospitalization, massive intraperitoneal bleeding due to rupture of the mass was observed. An emergency laparotomy was carried out, and excision of a large, ruptured, cystic mass involving the greater gastric curvature was performed. Microscopy revealed a gastric leiomyoblastoma. Even if infrequent, massive intraperitoneal bleeding is a very serious complication of gastric leiomyoblastoma. Considering the difficulty of an accurate preoperative diagnosis and the risk of intraperitoneal rupture, the authors suggest that similar abdominal masses should be managed by quick diagnostic investigations and early surgical procedures.