Angiomyoma of the hand: a post-traumatic tumor?

A 69-year-old woman presented with a tumor of the lateral aspect of the proximal phalanx of the little finger of the right hand. The finger was injured by a ring during a handshake. Excision and histopathological examination revealed the diagnosis of angio-myoma, a benign vascular tumor originating from the smooth muscle cells of arterial or venous walls. Angiomyomas belong to the family of leiomyomas and rare, small tumors (less than one centimeter), preferentially occurring in women between the ages of 40 and 60 years. They may be painful. Only one case of sarcomatous degeneration has been described in the literature. Trauma has never been previously reported as a cause of angio-myoma, but in this case the traumatic origin of the tumor was not in doubt.     

Giant cell tumor of bone: Is curettage the answer?

Background:

Giant cell tumors (GCT) are neoplasms of mesenchymal stromal cells with varied manifestations. There is no uniform accepted treatment protocol for these tumors,

Materials and Methods:

49 cases of proven giant cell tumors of appendicular skeleton, 27 prospective and 22 retrospective constituteed this study. The retrospective cases were collected by using computerized data base collection method. The patients were evaluated clinically, radiologically and by histology. Companacci grading and Enneking staging was used in the study. Two treatment modalities were used a) extended curettage (with/ without bone grafting/ cementation) or b) wide excision and reconstruction with a prosthesis or arthrodesis. Functional evaluation was done by Enneking''s system. Chi square tests, mann-whitney test and ANOVA were used for statistical analysis.

Results:

The average age was 26.82 years (16-50 years). 25 patients (51%) were recurrent GCT at presentation. The commonest site was lower end of femur (16 cases, 32.65%) and upper end of tibia (13 cases, 26.53%). 40 (81.63%) tumors had less than 5 mm of subchondral bone free of tumor. 35 (71.43%) tumors were Enneking''s surgical stage III and companacci grade III. Pathological fractures were seen in 12 (24.49%) cases. Intra-lesional currettage was used in 28 and enbloc excision in 19 patients and 2 (4.08%) underwent amputation. The average follow up period was 18.6 months (range 2-84). One recurrence was seen in a grade III recurrent distal radial lesion in the intralesional curettage group (3.57%) Enneking''s functional score with intralesional curettage (25.41) was better than enbloc excision (21.37). Enbloc excision had higher rates of infections (36.84 % Vs 25%) and soft tissue coverage problems (21.05% Vs 0).

Conclusion:

Intralesional therapy has a better functional outcome and less complications than enbloc excision, albeit with a high recurrence rate which can however be effectively treated with repeat extended curettage.     

Sertoli cell proliferations of the infantile testis: an intratubular form of Sertoli cell tumor?

We report on six boys with intratubular Sertoli cell proliferations (ISCPs), studied by routine histologic methods, electron microscopy, and immunohistochemistry of anti-müllerian hormone (AMH), inhibin alpha-subunit, 3beta-hydroxysteroid dehydrogenase (3beta-HSD), proliferative cellular nuclear antigen, and p53, and carefully followed for extended periods with periodic clinical examinations, testicular ultrasonographies, and determinations of serum levels of AMH and inhibin B. Peutz-Jeghers syndrome was found in four of six patients, and gynecomastia occurred in five of six patients. One boy had isosexual pseudoprecocity. ISCPs were observed as multiple foci of seminiferous tubules with large and proliferated Sertoli cells replacing germ cells and limited by the basement membrane. Mitotic figures, atypia, and/or interstitial invasion were not observed. Bilateral ISCPs were the only pathologic finding in three patients (patient nos. 1-3) and were associated with a microscopic tumor that resembled a large-cell calcifying Sertoli cell tumor (LCCSCT) in a fourth patient (patient no. 4). In the two remaining patients (patient nos. 5 and 6) ISCPs and LCCSCT were found in both testes. Ultrastructural examination showed large Sertoli cells, with round nuclei, sparse organelles, and some glycogen. Inhibin alpha-subunit immunolocalization was positive in the five patients in whom it was determined (patient nos. 2-6), AMH was positive in those ISCPs associated with tumors (patient nos. 4-6) and negative in isolated ISCPs (patient nos. 2 and 3); 3beta-HSD and PCNA were variable, and p53 was negative in all ISCPs. Patient nos. 1-4 have been followed for 2-19 years. One of them is currently entering puberty, the other two have already completed puberty and have testes of normal size, and the remaining one is an adult with clinically normal testes and sperm production. None of these patients had evidence of tumor development during follow-up as shown by serial ultrasonographies and serum levels of AMH and inhibin B. Patient nos. 5 and 6 who had bilateral ISCPs and LCCSCT were orchidectomized and evolved for 2-10 years after surgery without tumor recurrence. The prognostic significance of ISCPs, particularly when they are the only pathologic finding in a testicular biopsy, is a matter of controversy. Based on the long normal evolution, we recommend a conservative approach to therapy. The bilateral and multicentric character of ISCPs and their association with Sertoli tumors and Peutz-Jeghers syndrome suggest that they represent either proliferative lesions with tumorigenic potential or the intraepithelial stage in the evolution of some testicular Sertoli cell tumors.     

Dendritic cell infiltration in a patient with seminomatous germ cell tumor of the testis: is there a relationship with infertility and tumor stage?

PURPOSE: Inflammatory cells, such as dendritic cells, are considered to trigger the antitumoral immune response against tumors, such as testicular cancer. Male infertility associated with cancer may be due to endocrine or immunological factors. We investigated possible associations of antigen expression with dendritic cells, histiocytic cells and seminoma stage as well as with impaired spermatogenesis. MATERIALS AND METHODS: From 1992 to 1999, 30 patients with seminoma underwent orchiectomy at our center, including 14 who underwent spermiography before orchiectomy. Streptavidin-biotin immunostaining was performed on paraffin -embedded tumor specimens using antibodies against protein S-100 for dendritic cells and CD68-KP1 antigen. RESULTS: Light infiltration by less than 20 dendritic cells and less than 103 CD68+ cells was associated with tumor size greater than 1.5 cm. in 75% and 80% of patients, respectively. Strong infiltration by greater than 20 dendritic cells and greater than 103 CD68+ cells was associated with negative lymph nodes in 86% of patients. Slight infiltration by dendritic cells was observed in 71% of patients with a sperm count of greater than 8.6 million per ml. and in 100% with more than 45% motile sperm (p not significant and 0.02, respectively). Necrospermia increased with dendritic and CD68+ cell infiltration. No association was noted among preoperative serum tumor marker levels, the sperm count and immunostaining. CONCLUSIONS: Sperm autoimmunity is a plausible mechanism of infertility in men with germ cell tumors. Dendritic cells may induce antitumor cell cytotoxic reactions, but may also be cytotoxic to sperm cells or lead to inhibited spermatogenesis. Further studies focusing on tumor rejection antigen and the cloning of specific cytotoxic T lymphocyte against gametes are required to confirm these finding.     

Innate helper cells: a novel cell type essential in the initiation of asthma?

The role of the innate immune system in asthma initiation is being increasingly recognised, and several innate epithelial cytokines, such as interleukin 33 (IL-33), IL-25 and thymic stromal lymphopoietin, have been described as important in asthma pathogenesis. However, until now, the mechanism by which these cytokines initiate Th2 responses and form a link with the adaptive immune system was undetermined. The recent discovery of a new group of non-T, non-B innate helper cells, which are induced by epithelial innate cytokines and secrete the Th2 cytokines IL-4 and IL-13, may provide a mechanism by which the innate and adaptive immune systems become activated in asthma.     

A clear cell tumor of the lung presenting as a rapidly growing coin lesion: is it really a benign tumor?

There are few reported cases of clear cell tumor of the lung, a very rare benign mesenchymal neoplasm. We describe a 41-year-old asymptomatic man who presented with a coin lesion in a routine chest roentgenogram that was absent in a roentgenogram performed a year earlier. After a thorough workup, including radionuclide scintigraphy, the diagnosis of cell tumor of the lung was established. Somatostatin receptor positivity was demonstrated, and this clear cell tumor of the lung had a rapid growth rate. Because of these features, the benign nature of such a tumor remains questionable.     

Juxtaglomerular cell tumor of the kidney—a new classification scheme

ObjectiveTo introduce a new classification scheme of juxtaglomerular cell tumor (JCT) of the kidney for differential diagnosis of hypertension and renal cell carcinoma.MethodsFive cases of JCT have been diagnosed and treated surgically in our hospital during the last 4 years. Through a search in PubMed, we incorporated 7 large series of case reports of JCT into a review of 71 cases previously published in the literature. Clinical presentations (blood pressure), laboratory examinations [serum potassium, plasma renin activity (PRA), aldosterone (ALD), and renal venous sampling for renin assay], and imaging examinations [ultrasonography, computerized tomography (CT), excretory urography, and selective renal angiography] were summarized.ResultsThe 71 cases of JCTs can be classified into 3 types, which are typical type, atypical type, and non-functioning type. The 57 typical cases had the typical characteristics of hypertension, hypokalemia, hyperaldosteronism, and high renin. The 12 atypical cases had hypertension with normal potassium levels, and the 2 non-functioning cases had normal blood pressure and normal potassium levels.ConclusionsThe classification of typical, atypical, and non-functioning JCTs depends on blood pressure and serum potassium. JCT of the kidney should be considered in patients with hypertension and renal tumor, and nephron-sparing surgery is the first choice.     

Acinar cell cystadenoma of the pancreas: a new entity?

This report describes a newly observed cystic lesion of the pancreas showing acinar cell differentiation. The patients affected by this lesion included seven women and three men (age range 16-66 years). In six patients, all of whom were female and all but one of whom suffered from abdominal pain, the cystic lesions (diameters, 4-15 cm) were detected by imaging techniques and subsequently removed. In four patients the cystic lesions were incidental findings. Eight lesions occurred as unifocal, unilocular or multilocular cysts in the head (n = 6) or tail (n = 2) of the pancreas. One lesion was bifocal (head and tail) and another involved the entire pancreas. The cysts were only rarely connected with the pancreatic duct system, but with acinar structures. Their lining cells expressed pancreatic enzymes and lacked any cellular atypia or proliferative activity (Ki67 index <1%). For a follow-up period of 6-84 months all patients remained alive and well. Although a nonneoplastic nature cannot be fully excluded, we propose that this lesion, composed of well-differentiated acinar cells, may represent the benign counterpart of the well-recognized acinar cystadenocarcinoma. We therefore suggest the term acinar cell cystadenoma.     

Determination of pyruvate kinase type tumor M2 in human renal cell carcinoma: a suitable tumor marker?

Renal cell carcinoma (RCC) expresses an isoform of the glycolytic enzyme pyruvate kinase (type M2). The dimeric form (TuM2-PK) is over expressed in tumor cells and is detectable in blood with a sensitive enzyme-linked immunosorbent assay (ELISA). The aim of the present study was to evaluate the clinical value of TuM2-PK as a tumor marker for RCC. The TuM2-PK concentration in EDTA-plasma was determined quantitatively and immunologically using an ELISA. We measured the TuM2-PK plasma levels of 83 patients before and after surgery. Ninety-seven patients with various non-malignant diseases were also recruited as a control group. The control group displayed mean levels of 11.37 U/ml of TuM2-PK. Values were elevated in patients with RCC prior to surgery (mean 21.88 U/ml). The plasma levels increased after surgery until day 5 (mean 53.97 U/ml). At day 10, marker levels started to decrease without reaching preoperative values (mean 43.5 U/ml). Plasma levels in the renal vein (obtained during surgery) were not different from those in the peripheral blood. Follow-ups after 2-6 months showed a decrease to below preoperative levels (mean 16.3 U/ml). A significant difference was obtained by comparing the patients according to their Robson score. We found a significant difference (P < 0.01, Wilcoxon's two-sample test) in TuM2-PK levels between patients with RCC and the control group. Nevertheless, using the manufacturer's recommended cut-off value (15 U/ml), sensitivity was only 50.6% and specificity was 80.4%. Our results suggest that TuM2-PK is not a suitable tumor marker for RCC.     

Hürthle cell tumor of the thyroid: analysis of 188 cases

We reviewed 188 cases of Hürthle cell tumor of the thyroid (HCT) between 1982 and 1996. There were 160 women and 28 men with a mean age of 51.8 years. Thirty-one of the patients had cancer, and the others had adenoma. Age, size of the primary tumor, and preoperative thyroglobulin level were not significantly different in the cancer and adenoma patients. The gender ratio, however, was significantly different (p < 0.05). Recurrent HCT was observed in three patients with adenoma. Two patients had subcutaneous recurrence (suspected implantation), and the other patient had recurrence in the residual thyroid gland. All patients with recurrence of adenoma underwent partial lobectomy at the initial operation. Three cancer patients had recurrent disease. Locoregional recurrence was observed in one patient and distant metastases in two patients (lung in one, lung and bone in one). One of the patients with distant metastasis died from the disease, and the other is alive with the disease. Tumor implantation was observed in patients with adenoma, so intraoperative handling of the tumor requires care. It also means that this tumor, even though benign, is aggressive in terms of proliferative activity. All patients with Hürthle cell tumor should be treated by total lobectomy at least. The outcome of the cancer patients was not as poor as in previous reports.     

Wilms tumor—a renal stem cell malignancy?

Wilms’ tumor (WT; nephroblastoma) is the most common pediatric renal malignancy and rated fourth in overall incidence among childhood cancers. It is viewed as a prototype of differentiation failure in human neoplasia as it recapitulates the histology of the nephrogenic zone of the growing fetal kidney. The cellular origin of WT is unclear. However, recent genomic, genetic and epigenetic studies point to an early renal stem/progenitor cell that undergoes malignant transformation as the source for WT. In this context, classical WT shares genes and pathways activated in progenitors committed to the renal lineage. However, direct proof and characterization of the WT initiating cell have remained elusive. Novel methodologies recently adopted from the cancer stem cell scientific field, including the analysis of sorted single human tumor cells, have been applied to WT. These have enabled the identification of cell sub-populations that show similarities—in terms of molecular marker expression—to human fetal kidney progenitors and are, therefore, likely to be derivatives of the same lineage. Further elucidation of the WT cancer stem cell or the cell of origin in human tumors and in transgenic mouse models that generate murine tumors may not only provide novel therapeutic targets but also shed light on the normal kidney stem cell.     

Squamous cell carcinomas in children and young adults: a new wave of a very rare tumor?

Background

Squamous cell carcinomas (SCCs) seem to have become more common recently. This study aims to check whether the increase is real and to review possible etiologic factors and problems in diagnosis.

Methods

Patients with SCC were identified from anatomical pathology files over 30 years. The number and primary sites seen in the first 15 years were compared with those in the second. Histories were reviewed for predisposing factors. Mucosal tumors were tested for human papillomavirus (HPV) and Epstein-Barr virus by polymerase chain reaction.

Results

One cutaneous SCC and 2 nasopharyngeal carcinomas (NPCs) were seen in the first period, and 2 cutaneous SCCs and 3 nasopharyngeal carcinomas in the second. Another 9 patients with mucosal SCCs were seen in the second period, many with history of cancer treatment or immunosuppression. Two laryngeal SCCs were HPV16-positive. Histologic diagnosis was difficult in 3 patients.

Conclusion

Squamous cell carcinomas have become more common in the last 15 years. Causes include improved survival of cancer patients, therapeutic irradiation, immunosuppression, and possibly, increased prevalence of HPV in the community. Awareness of this increase in children, early biopsy in susceptible patients, repeat wider biopsy, and consultation with adult pathologists may reduce the diagnostic delay.     

NKT cells: the culprits of sepsis?

Sepsis is currently a leading cause of death in hospital intensive care units. Previous studies suggest that the pathophysiology of sepsis involves the hyperactivation of complex pro-inflammatory cascades that include the activation of various immune cells and the exuberant secretion of pro-inflammatory cytokines by these cells. Natural killer T-cells (NKT) are a sub-lineage of T cells that share characteristics of conventional T cells and NK cells, and bridge innate and adaptive immunity. More recently, NKT cells have been implicated in microbial immunity, including the onset of sepsis. Moreover, apolipoprotein E (apoE), a component of triglyceride-rich lipoproteins, has been shown to be protective in endotoxemia and gram-negative infections in addition to its well-known role in lipid metabolism. Here, we will review the role of NKT cells in sepsis and septic shock, the immunoregulatory role of apoE in the host immune response to infection, and propose a mechanism for this immunoregulation.     

An unusual tumor of rib diaphysis—report of a giant cell tumor and a brief review of literature

Giant Cell Tumor of bone (GCT) is a benign but aggressive tumor, which forms about 4?C5% of primary bone tumors and 1?C2% of all chest wall tumors. It arises in the epiphysis of bones. The epiphysis of a rib is in its head and tubercle posteriorly and hence a GCT arising in a rib??s anterior aspect, its diaphysis, is rare. In this unusual position, it can be mistaken for other more common diaphyseal pathologies. Radiological images are often diagnostic. A needle biopsy is best avoided and a wide excision biopsy is the treatment of choice. Microscopically, multinucleated giant cells are seen amidst stromal cells. Giant cells like these are also seen in other diseases like the brown tumor of primary hyperparathyroidism. Giant cell lesions are never caused by secondary hyperparathyroidism. We present a case of a diaphyseal GCT of rib in a patient with secondary hyperparathyroidism who was successfully treated.     

Solid-pseudopapillary tumor of the pancreas: A surgical enigma?

BACKGROUND: Solid-pseudopapillary tumors (SPTs) of the pancreas have been reported as rare lesions with "low malignant potential" occurring mainly in young women. This study was designed to define the clinicopathological characteristics and the effect of surgical intervention. METHODS: A retrospective review from January 1985 to July 2000 was performed. Clinicopathological, operative, and survival data were obtained. The Kaplan-Meier method and chi2 analysis were performed. All cases were re-reviewed by a senior pathologist. RESULTS: During this time, 24 patients were diagnosed as having SPTs (0.9%). Twenty females and four males were identified, with a median age of 39 years (range, 12-79). The median size of the lesions was 8.0 cm (range, 1-20). Two patients' tumors were found to be unresectable at initial presentation because of vascular invasion; both patients have remained alive with disease, one for 13 years and the other 1 year. At a median follow-up of 8 years, one recurrence occurred in 17 patients who underwent complete resection. Microscopic margin positive (P = .26), invasion of surrounding structures (P = .51), and size >5 cm (P = .20) were not significant predictors of survival. Four patients presented with synchronous liver metastasis and underwent resection of the primary tumor and the liver metastasis, with one patient dying of progression of metastatic disease at 8 months, another alive with recurrence in the liver at 6 years, and the last two alive without evidence of disease at 1 month and 11 years. CONCLUSIONS: SPT occurs predominantly in women (82%), although it can occur in men; all age groups are affected. Complete resection is associated with long-term survival even in the presence of metastatic disease.     

Histologic variants of renal cell carcinoma: does tumor type influence outcome?

Each histologic type of renal cell carcinoma (RCC) has different pathologic and clinical parameters; however, the independent role of histologic type in outcome prediction remains contested. Most studies show relevance for outcome of each histologic type when correlated with survival by univariate analysis, whereas few studies show differences in outcome once other key prognostic factors, such as stage and grade, are considered. These studies highlight the challenges to prove outcome relevance. Despite the contested independent value of type for outcome prediction, separation of RCC into types is well accepted and can be substantiated on clinical, pathologic, molecular, and general outcome differences.