Malignant lymphoma obscured by concomitant extensive epithelioid granulomas: report of three cases with similar clinicopathologic features

Three similar cases are described of an unusual combination of malignant lymphoma and extensive non-necrotic granulomas. The three patients presented with prominent splenomegaly without peripheral lymphadenopathy. They had normal or moderately elevated lymphocyte counts, abnormal lymphoid cells in the peripheral blood and bone marrow, and abnormalities of serum immunoglobulins. The lymphoid tumor was difficult to recognize but it was best identified in abdominal lymph nodes, it was composed of small atypical lymphocytes proliferating in a vaguely nodular pattern. The presence of multiple epithelioid granulomas obscured the neoplastic proliferation in the spleens and misled or delayed the final interpretation of the malignant disease. Abdominal lymph nodes and liver also contained granulomas although to a lesser extent. Studies of the lymphocyte surface characteristics in one patient suggested that the neoplasm derived from a monoclonal proliferation of B cells. The relationship between the exuberant epithelioid granulomas and the underlying neoplastic lymphoid proliferation is not clear. Regardless of whether it represents a distinct clinicopathological entity, recognition of this remarkable association has important practical implications since the lesions may be erroneously interpreted by the pathologist.     

Cervical adenitis: report of two cases

OBJECTIVES: A recent review or meta-analysis of epidemiologic studies concluded that persons with asbestos-related pleural plaques do not have an increased risk of lung cancer in the absence of parenchymal asbestosis. The reviewer inferred that this conclusion provided indirect supportive evidence for the proposition that asbestosis is a necessary precursor of asbestos-related lung cancer. The objective of the present communication is to contest these claims. METHODS: Finnish epidemiologic data and population statistics were used to estimate the apparent risk ratio of lung cancer associated with radiographic signs of pleural plaques. Power calculations were applied to compute the needed population sizes to demonstrate that the association is statistically significant. RESULTS: Unrealistically large population studies would be needed to observe the statistical relation between pleural plaques and lung cancer, quantitated as a risk ratio of 1.1, resulting from relatively low levels of environmental asbestos exposure. In realistic and valid epidemiologic studies on heavily exposed subpopulations, a two- or threefold risk can be identified. CONCLUSIONS: Uninformative studies should not be interpreted as providing suppressive evidence that pleural plaques are a noncausal risk indicator of lung cancer. Even for the null hypothesis, the inference that asbestosis is a necessary causal link between asbestos and lung cancer is illogical.     

Fiberglass dermatitis: report of two cases

Fiberglass is widely used for insulation and as a reinforcement filling material. Handling fiberglass products may induce contact dermatitis. We report on the first two cases of fiberglass dermatitis reported in Taiwan. The first patient suffered from a severe pruritic eruption two hours after repairing a roof with wave-form ceiling boards. Erythematous maculopapules were present on both hands and finger webs. The second patient was a quality controller of printed circuit boards (PRCBs). She presented with erythematous maculopapules on the face and excoriated papules and lichenified plaques on the trunk and forearms, which had been present for two years. Scrapings of the skin lesions from both patients showed fiberglass spicules of 7.5 to 8 microns in diameter. Similar fibers were detected in scrapings from the wave-form ceiling board and PRCB. Histopathology of the second case revealed spongiotic psoriasiform dermatitis. Patch tests in case 2 with the plastics and glues series, epoxy resin and scrapings from the PRCBs were all negative. Fiberglass dermatitis may be easily misdiagnosed. Clinically, it may resemble scabies, eczematous dermatitis, folliculitis, petechiae and urticaria. A high index of suspicion is essential for a correct diagnosis.     

Ameloblastic fibro-odontoma: report of two cases

Ameloblastic fibro-odontoma is a rare odontogenic tumour. It affects young people and is usually located in the posterior jaws. Two cases of this tumour are presented: both lesions had produced an asymptomatic swelling. The histology showed the presence of an odontogenic epithelium immersed in a fibrous mesenchyme; dental hard tissues were also present. No recurrences were found after 12 and 4 years from the surgical enucleation of the tumour.     

Leiomyoma of the bladder: report of two cases

We report 2 cases of leiomyoma of the urinary bladder. A 41-year-old female visited our hospital with the complaint of pollakisuria. A solid tumor of the urinary bladder was found by ultrasonography. A large shadow defect at the left-anterior wall was shown by drip infusion pyelography (DIP). Computed tomographic scan (CT) and magnetic resonance imaging (MRI) also revealed a large tumor. T1-weighted image revealed a homogeneous low intensity tumor and T2-weighted image disclosed heterogeneous low intensity tumor. Cystoscopy revealed a large submucosal tumor. Partial cystectomy was performed, and she has had neither recurrence nor metastasis for 36 months. A 32-year-old male was referred to our hospital with the complaint of macrohematuria. A solid tumor of the urinary bladder was found by ultrasonography. A shadow defect was not clearly detected by DIP. A large tumor was detected on the anterior wall by MRI. T1-weighted image showed a homogeneous low intensity tumor and T2-weighted image disclosed a high intensity tumor. Cystoscopy revealed a submucosal tumor on the anterior wall. Urine cytology did not suggest a malignancy. The biopsied specimens revealed only an inflammatory change in the mucosa. Partial cystectomy was carried out. He has had neither recurrence nor metastasis for 29 months. Histological diagnosis in both cases was leiomyoma of the urinary bladder.     

Malignant duodenocolic fistula: report of two cases, each with one or more other synchronous gastointestinal cancers

Two patients had duodenocolic fistulas, each following a carcinoma of the colon in the area of the hepatic flexure that had perforated into the duodenum. The first patient was treated by a radical pancreatoduodenectomy with right colectomy; the second by subtotal colectomy with excision of the duodenal wall and suture. Both patients are alive and without evidence of recurrent disease. In addition, the first patient had two other primary carcinomas, in the cecum and in the stomach, and the second patient had another primary in the sigmoid. The definitive procedure had to be adjusted to encompass all lesions. The radical operation in one stage seems to be the preferred procedure and certainly is most satisfactory as a cancer operation. Our patient treated by this procedure has survived more than 11 years. An intestinal fistula related to colonic carcinoma, evan though rare, should not be considered as a separate entity. Treatment of the cancer with an en-bloc resection of the communicating organs should be employed if possible.     

Pyothorax-associated lymphoma: description of the first two cases detected in Italy

BACKGROUND: Pyothorax-associated lymphoma (PAL) is a rare, but distinct, clinico-pathologic entity which occurs most often in Japanese people; to the best of our knowledge, only six cases of it have been reported in Western countries. The tumour develops several decades following artificial pneumothorax or chronic pleuritis due to tuberculous infection, produces pleural effusion associated with extensive local lymphomatous infiltrates, and is sustained by a polymorphic large B-cell clonal proliferation showing EBV integration in the genoma of the neoplastic cells. PATIENTS AND METHODS: Herein we describe two cases of PAL observed in Italian patients, both extensively studied on the clinical, pathological, phenotypic, virological, and molecular levels. RESULTS: The two cases occurred, respectively, 45 and 50 years after therapeutic pneumothorax because of tuberculous pleuritis and were characterized by a pleural mass extending to the thoracic wall, which on histological examination were seen to consist of large elements with immunoblastic morphology. Immunohistochemistry show monotypic restriction of Ig light chains, as well as the expression of CD45, B-cell markers (CD20, CD79a, CD45RA), bcl-2 oncogene product, EBNA-2 and, partially, LMP-1. The ratio of cycling cells was extremely high as was the number of mitotic figures. In situ hybridization displayed the presence in the neoplastic cells of the EBV-related small RNAs EBER 1 and 2, which in turn, along with the positivity for EBNA-2 and LMP-1, further strengthened the close relationships between PAL and latent viral infection. Molecular studies revealed, on one hand, clonal rearrangement of the Ig heavy chain J region genes, and on the other, negativity for HHV8 in one case and positivity in the other. CONCLUSIONS: These cases of PAL are the first to be documented in Italy; they serve to direct attention to the fact that this condition is not confined to Japanese people, and that its occurrence in Western countries might be underestimated.     

Pagetic sarcoma of the calvarium: report of two cases

DNA-based mutation analysis on the connexin 32 gene was performed in 49 families with Charcot-Marie-Tooth disease (CMT) type 1 but without duplication involving the chromosomal region, 17p12-p11.2. Mutations were identified in five of the 49 families, and four of the five mutations were hitherto undescribed: Va137Met, Glu57His, Arg142Glu, Val177Ala. X-linked CMT sometimes lacks evidence for X-linked transmission and cannot be differentiated from CMT type 2, especially in females with mildly decreased nerve conduction velocity. Therefore, molecular analysis is useful for molecular pathology of their disease.     

Malignant histiocytosis-like B-cell lymphoma, a distinct pathologic variant of intravascular lymphomatosis: a report of five cases and review of the literature

Malignant histiocytosis (MH)-like B-cell lymphoma (BCL) is a neoplastic proliferation of large B cells clinically characterized by fever, hepatosplenomegaly, haemophagocytosis and abnormal laboratory data, without lymphadenopathy or skin lesions. Interestingly, most cases have been reported in Asian patients, and it is unclear whether MH-like BCL is biologically distinct from conventional large B-cell lymphomas. We report five Japanese patients with MH-like BCL. Biopsied specimens of bone marrow, liver and/or spleen showed infiltration of neoplastic B cells accompanied by haemophagocytosing histiocytes. Lymphoma cells were positive for CD19, CD20 and HLA-DR surface antigens, and negative for CD5 and CD10. In four cases elevated serum levels of interleukin (IL)-6 and the soluble IL-2 receptor isoform were noted, but not IL-1beta, IL-2 or tumour necrosis factor-alpha. Autopsies of two cases were pathologically diagnosed as intravascular lymphomatosis (IVL). Based on these observations, the current and nine previous cases reported as MH-like BCL in Japan were re-evaluated. They appear to form a peculiar variant of IVL, characterized by bone marrow involvement at presentation, haemophagocytic syndrome, and a rapidly aggressive clinical course, but rarely neurological complications or skin lesions. This variant may merit separate consideration because of the problems posed in the initial diagnosis and therapeutic approaches.     

Werdnig-Hoffmann disease: report of two cases?]

Two cases of familial spinal muscular atrophy with onset in infancy are reported. The author makes an attempt to categorize them and concludes by Werdnig-Hoffmann disease.     

Mediastinal cystic lymphangioma: report of two cases

Cystic lymphangioma is an extremely rare mediastinal benign tumor characterized by congenitally enlarged lymphatic vessels. It is often diagnosed incidentally because it is only symptomatic when it grows to be very large. We present two cases of mediastinal cystic lymphangioma and review the literature.     

Synovial sarcoma of the vulva: a report of two cases

We report two cases of synovial sarcoma arising in the vulva. The patients were 30 and 37 years old and presented with a painless mass that was interpreted clinically as a cyst. The tumors were 2.0 and 1.2 cm in greatest diameter. Histologically, they were composed of epithelial cells forming solid nests and gland-like and papillary structures surrounded by spindle-shaped cells. Immunohistochemically, the epithelial cells stained for cytokeratin and the spindle-shaped cells for vimentin. Ultrastructurally, the epithelial cells had prominent intercellular junctions and narrow microvilli and were separated from the spindle-shaped cells by a basal lamina. The spindle-shaped cells were closely apposed with focal intercellular contacts. One tumor recurred locally 3.5 years after excision, but the patient was alive and well 1 year after a re-excision and radiation therapy. The other patient was alive and well 4 years after an excision. These tumors are the first reported examples of synovial sarcoma arising in the vulva.     

Wernicke's encephalopathy and dialysis: report of two cases

Wernicke encephalopathy is considered a complication of dialytic therapy, but there are few reports of this complication. We report a 57 years old man and a 45 years old woman, with grade IV renal failure, who after acute peritoneodialysis and chronic hemodialysis respectively, had a confusional syndrome that responded to the administration of thiamine. CT scans in both patients discarded abnormal blood collections or new cerebrovascular episodes. The man bad two previous cerebrovascular episodes, a severe anemia that was corrected, angina and an episode of arrhythmia during the dialytic procedure previous to the confusional episode. The woman had an acute uremic syndrome and a concomitant urinary tract infection during the confusional episode. Wernicke encephalopathy must be suspected in patients in dialysis with confusional episodes.     

Spontaneous spinal extradural hematomas: report of two cases

INTRODUCTION AND CLINICAL CASES: We present two cases of spontaneous spinal extradural hematoma, which occurred in 1996 and were admitted to and operated on by our Department. Both patients were women. No cause for the bleeding was found in either case. Both presented with the site of the lesion in the thoracic spine, although at different levels. There was acute onset of the clinical condition. The best diagnostic procedure was MR. The treatment of choice was surgical and the results related to the rate of onset of the clinical picture and the time elapsed before operation. CONCLUSION: The form of presentation is discussed, together with the importance of early diagnosis and the relation to emergency surgical treatment.     

Enamel pearls in the primary dentition: report of two cases

Concentrations of the polyamines, putrescine, spermidine and spermine were investigated in rat brains, in which chemical kindling or single convulsion had been induced by intraperitoneal injection of pentylenetetrazol (PTZ). A single injection of 60 mg/kg of PTZ produced tonic-clonic convulsion and increased the putrescine concentration 8 h after the injection. At lower doses of PTZ (10 and 30 mg/kg), neither marked behavioral seizure nor significant change in any polyamine concentration was observed. On the other hand, repeated injections of 30 mg/kg of PTZ eventually resulted in intense motor seizures (PTZ kindling) and increased the concentrations of all three polyamines. The most marked increase was detected in putrescine 1-48 h after the intense seizures. The increase in putrescine was clearly higher in PTZ kindling than in single convulsion. These results suggest that increases in polyamine concentrations are involved in neuronal excitability in the epileptic brain.