Hairy cell leukemia associated with carcinoma: report of two patients and review of the literature

The association of carcinoma and hairy cell leukemia (HCL) in two patients is recorded. One of the cases was a 58-year-old male who developed carcinoma of the kidney, while the second patient was a 48-year-old woman with carcinoma in the breast. This rare association is probably coincidental, as it is not described in most of the larger reported series of patients with HCL. It is of interest to note that the first patient had received radiation therapy thirty years before the diagnosis of HCL and carcinoma was made.     

Inhibin concentrations in mares with granulosa cell tumors

The hormone-producing equine granulosa cell tumor (GCT) may secrete high levels of inhibin. Measurement of inhibin concentrations may be useful in the diagnosis and conformation of mares with GCT. Inhibin may be measured using RIA, which recognizes dimeric alphabetaA-inhibin as well as the monomeric (free) inhibin alpha-subunit, or using a two-site immunoradiometric assay (IRMA) specific for alphabetaA-inhibin. The objective of this study was to examine concurrent relationships among alpha-inhibin (as measured using RIA), alphabetaA-inhibin (as measured using IRMA), and other hormones (testosterone, estradiol, LH, FSH) in mares with GCT. Hormone concentrations were measured in single serum or plasma samples obtained from 22 mares with GCT and from 31 normal cycling mares. One GCT mare had blood samples collected at 12-h intervals for 21 days, and at 15-min intervals for two 6-h periods during that time. Results showed that in GCT mares alpha-inhibin was increased to a greater extent, was more uniformly elevated, and had a less variable secretory pattern than did alphabetaA-inhibin. Concentrations of alpha-inhibin and tumor mass were positively correlated (P < 0.01). Concentrations of LH were higher (P < 0.02) in GCT mares than control mares and were positively associated with testosterone concentrations (P = 0.05). Concentrations of FSH tended to be lower in GCT than control mares and were inversely related with alphabetaA-inhibin in GCT mares. Testosterone and estradiol concentrations were variable. It was concluded that immunoreactive alpha-inhibin reflected detection of both alphabetaA-inhibin and free a-subunit. Free alpha-subunit was evidently secreted at a relatively steady rate dependent upon mass of the GCT, whereas secretion of alphabetaA-inhibin was more responsive to FSH regulation. Determination of alpha-inhibin using RIA appeared to be a more reliable indicator of the presence of a GCT than specific measurement of alphabetaA-inhibin using IRMA.     

滤泡树突细胞肉瘤二例报道并文献复习

目的探讨滤泡树突细胞肉瘤（FDCS）的临床表现、病理形态、免疫组化、生物学行为和预后,提高对该病的认识。方法对2例滤泡树突细胞肉瘤进行报道,并结合临床资料、肿瘤大体及镜下特征、免疫组化标记结果及预后和国内外文献进行分析。结果2例FDCS,1例发生于颈部淋巴结,另1例发生于肠系膜,镜检肿瘤由梭形及卵圆形细胞组成,呈束状、漩涡状、编织状排列;免疫组化显示肿瘤细胞CD21、CD23和CD35阳性;患者预后不良。结论滤泡树突细胞肉瘤是一种罕见且容易误诊的低度恶性肿瘤,组织化学和免疫组化染色有助于该肿瘤的诊断及鉴别诊断,减少误诊。     

Primary malignant melanoma of the nasal cavity: report of two cases and review of the literature

Primary malignant melanomas of the nasal cavity are rare, as only 400 cases have been reported to date. The present paper describes two cases recently seen in Caucasian women. The authors point out the difficult clinical diagnosis, as the symptoms are rather aspecific. From the histopathological point of view, diagnosis is easy in the melanotic cases while can show interpretating problems in the amelanotic ones, when melanoma is almost indistinguishable from other malignant neoplasms. A correlation between histological grading and prognosis was not detected, as both cases showed local recurrences within one year after surgery although they were, respectively, of epithelioid and undifferentiated type. While surgery appears to be the choice treatment of the primary lesion, the treatment of cervical metastasis is still disputable. On the whole, most authors think that the role played by radio- and mainly chemo-therapy is still limited and that cervical adenopathies should be treated by a simple lympho-adenectomy rather than by a neck dissection.     

Gastrointestinal stromal tumors in a baboon, a spider monkey, and a chimpanzee and a review of the literature

Background  Gastrointestinal stromal tumors (GISTs) are believed to originate from the intestinal pacemaker cells (interstitial cells of Cajal) or their progenitor cells. Spontaneous tumors have been reported in dogs, horses, rhesus, and a chimpanzee and they have been produced experimentally in mice and rats. GISTs represent a diagnostic challenge because they cannot be differentiated from non-lymphoid mesenchymal tumors without using human c-kit (CD117) immunohistochemistry.
Methods  Three neoplasms were incidental findings at necropsy in the stomachs of a baboon and a spider monkey and in the rectum of a chimpanzee.
Results  The GISTs were initially diagnosed grossly and histologically with hematoxylin and eosin as leiomyomas. Immunohistochemical analysis revealed that all three were c-kit (CD117) positive.
Conclusions  These are the first reports of GISTs in the baboon and spider monkey and the second in a chimpanzee. The occurrence of GISTs in non-human primates may provide a unique opportunity to study these tumors.     

Ring chromosome 10: report on two patients and review of the literature

Ring chromosome 10—r(10)—is a rare disorder, with 14 cases reported in the literature, but only two with breakpoint determination by high-resolution techniques. We report here on two patients presenting a ring chromosome 10, studied by G-banding, fluorescent in situ hybridization (FISH), multiplex ligation-dependent probe amplification (MLPA) and SNP-array techniques, in order to investigate ring instability and determine breakpoints. Patient 1 showed a r(10)(p15.3q26.2) with a 7.9 Mb deletion in 10q26.2-q26.2, while patient 2 showed a r(10)(p15.3q26.13) with a 1.0 Mb deletion in 10p15.3 and a 8.8 Mb deletion in 10q26.13-q26.3, both unstable. While patient 1 presented with clinical features usually found in patients with r(10) and terminal 10q deletion, patient 2 presented characteristics so far not described in other patients with r(10), such as Dandy-Walker variant, osteopenia, semi-flexed legs, and dermal pigmentation regions. Our data and the data from literature show that there are no specific clinical findings to define a r(10) syndrome.     

Cell-bound antiglial immunity in patients with malignant tumors of the brain

Peripheral blood lymphocytes from patients with malignant brain tumors were found to have a cytotoxic effect against cultured autologous tumor cells as well as normal adult and fetal glial cells obtained from 18- to 20-week surgical abortions. In a blind series of 32 patients, five of nine patients with a malignant glioma could be detected by using ⁵¹Cr-labeled fetal glial cells as targets and appropriate controls. Lymphocytes from patients with “benign” gliomas, nonglial, or metastatic tumors were characterized by a low or absent cytotoxicity.The results are interpreted to show a development of cell-bound immunity against normal glial antigens in patients with destructive infiltrating glial tumors carrying the antigenic determinants through the immunological barrier normally isolating the central nervous tissues.     

Steroid sulfatase activity in the rat ovary, cultured granulosa cells, and a granulosa cell line

Direct production of gonadal steroids from sulfated adrenal androgens may be an important alternative or complementary pathway for ovarian steroidogenesis. The conversion of sulfated adrenal androgens, present in serum at micromolar concentrations in adult women, into unconjugated androgens or estrogens requires steroid sulfatase (STS) activity. STS activity has not been characterized in the rat ovary. Substantial STS activity was present in homogenates of rat ovaries, primary cultures of rat granulosa cells, and a granulosa cell line, as determined by conversion of radiolabeled estrone sulfate (E₁S) to unconjugated estrone. The potent inhibitor estrone sulfamate eliminated the STS activity. Using E₁S as a substrate with microsomes prepared from a granulosa cell line, the K_m of STS activity was approximately 72 μM, a value in agreement with previously published data for rat STS. Therefore, ovarian cells possess STS and can remove the sulfate from adrenal androgens such as dehydroepiandrosterone sulfate (DHEA-S). Using DHEA-S as a steroidogenic substrate represents an alternative model for the production of ovarian steroids versus the “two cell, two gonadotropin” model of ovarian estrogen synthesis, whereby thecal cells produce androgens from substrate cholesterol and granulosa cells convert the androgens into estrogens. The relative contribution of STS activity to ovarian steroidogenesis remains unclear but may have important physiological and pathophysiological implications.     

Diagnosis and management of a malignant granulosa cell tumor in a holstein nulligravida: a case study

Transrectal palpation of an 18-mo-old, anestrous Holstein nulligravida revealed an enlarged left ovary. Ultrasonographically, the mass resembled known equine granulosa cell tumor (GCT) images. Slowly, virilism developed. Fifty-five days after initial diagnosis, unilateral ovariectomy was performed The presurgical serum concentration of testosterone was equal to that of a mature bull. During the 3 mo period following surgery, estrus expression resumed, the serum testosterone concentration returned to baseline, and the heifer was diagnosed pregnant following AI. A low grade, malignant GCT was the final histopathologically diagnosis. Clinical diagnosis of GCT should involve ultrasonography and steroid hormone assay. An ultrasound image, similar to the one shown, may be characteristic of GCT in the bovine.     

Second primary malignant tumors in patients with ocular melanoma

Multiple primary malignant tumors have been documented with increased frequency over the last two decades. Continuously increasing success of modern oncotherapy has led to long-term remissions in many cases, but this success rate poses a growing risk for the development of second primary malignancies. The incidence of those involving an intraocular tumor is relatively rare. In the present study we report five ocular melanoma patients with second primary malignant tumors diagnosed during a fourteen-year period in our department. We wish to emphasize that an intraocular mass lesion in a patient with a history of a previous malignancy should not be dismissed as a metastatic lesion. The diagnosis of an intraocular lesion as a separate primary tumor drastically changes the prognosis and the therapeutic approach.     

Hypertrichosis cubiti: two new cases and a review of the literature

Two cases of hypertrichosis cubiti in combination with short stature, facial dysmorphias and retarded development are reported with a review of the literature. Hypertrichosis cubiti, the hairy elbows syndrome, consists of a localized form of long vellus hair on the extensor surfaces of the distal third of the upper arm and the proximal third of the forearm bilaterally. It can be associated with short stature and other physical abnormalities. The mode of inheritance has not been established yet; an autosomal recessive as well as an autosomal dominant inheritance trait are postulated.     

In-vitro studies of granulocyte phagocytosis in patients with malignant tumors

Investigations are presented about granulocyte phagocytosis with rice starch in patients with clinically curable malignant tumours and with those treated palliatively. A significantly reduced granulocyte efficiency of phagocytosis indicating a bad prognosis of the disease can be found in those patients being in the advanced tumour stage.     

Primary hepatic malignant fibrous histiocytoma: a case report and review of the literature

Primary malignant fibrous histiocytoma (MFH) of the liver remains extremely rare with only several cases having been reported in literature. We report a case of hepatic MFH in a 53-year-old man who presented with upper abdominal pain, and weight loss for one month. Ultrasound and computed tomography (CT) scan showed a large mass with fine tumor vessels over the left lobe of the liver. Histopathological findings indicated a mesenchymal tumor consisting of spindle cells in storiform pattern intermingled with histiocyte-like cells and giant cells. Immunohistochemically, most tumor cells expressed vimentin, alpha-1 anti-chymotrypsin, alpha-1 antitrypsin and CD68. Morphological and immunohistochemical findings support that the tumor should be classified as a primary malignant fibrous histiocytoma. The literatures is briefly reviewed.