Intra-cardiac extension of a Wilms’ tumor

Background

Wilms? tumor, or renal nephroblastoma, is the commonest malignant renal tumor in children and young adults. Intravascular and intracardiac extension of this tumor is a very rare and challenging clinical entity.

Case presentation

The case is reported of a 6-year-old boy diagnosed with nephroblastoma, in whom the tumor involved the inferior vena cava and the right atrium. The successful multimodal surgical management consisted of combined, synchronous surgical resection of both renal and intracardiac tumors. The boy had an uneventful postoperative course and recovery, followed by relapse-free living.

Conclusion

Intra-cardiac extension of Wilm’s tumor poses a surgical challenge where the favorable outcome is provided only by the combined and well-coordinated, skilled multidisciplinary care of pediatric surgeons and pediatric cardiac surgeons.

     

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.     

Seminoma – a unique case of tumor located in the head of epididymis

We report a very rare case of tumor in the head of left epididymis without localized primary foci of tumor in the testis.     

'Spindle cell oncocytoma' of the adenohypophysis: a tumor of folliculostellate cells?

We describe five primary tumors of the adenohypophysis featuring mitochondrion-rich spindle cells. The patient ages ranged from 53 to 71 years (mean 61.6 years); two were female. All presented with panhypopituitarism. Two also had visual field defect. On neuroimaging all tumors showed suprasellar extension and were indistinguishable from pituitary adenoma. None showed imaging or operative evidence of dural involvement. All were gross totally removed: four by transsphenoidal surgery and one by frontal craniotomy. Follow-up ranged from 2 to 68 months (mean 35.4 months). No recurrences were noted. The clinical workup was noncontributory in all but two patients: one (case no. 4) with an oncocytic thyroid adenoma and another (case no. 5) with squamous carcinoma of both the uterine cervix and of vocal cord. Histologically, the five tumors were composed mainly of fascicles of spindle cells with eosinophilic, granular cytoplasm. Mitoses were rare and necrosis was absent. Neoplastic cells were immunoreactive for vimentin, epithelial membrane antigen, S-100 protein, and galectin-3. Stains for pituitary hormones, synaptophysin, chromogranin, glial fibrillary acidic protein, cytokeratin CAM5.2, smooth muscle actin, CD34, and CD68 were negative. No thyroglobulin immunoreactivity was noted in the tumor of case no. 4. Ultrastructurally, the neoplastic cells contained numerous mitochondria with lamellar cristae. The neoplastic cells were linked by intermediate junctions and desmosomes. No secretory granules were noted. The histologic, immunohistochemical, and fine structural features of these tumors were unlike those of pituitary adenoma or any other primary sellar tumor. A derivation from adenohypophyseal folliculostellate cells is suggested.     

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.     

Dentinogenic ghost cell tumor – Case report of a rare entity

IntroductionDentinogenic ghost cell tumor (DGCT) is an entity with about 60 cases reported in the literature. It is a benign odontogenic tumor, despite being locally invasive and associated with a risk of local recurrence.Presentation of caseA 47-year-old woman presented with a 2-year-old expansive bone lesion. Radiologically, a multilocular mass was identified in the left superior maxilla, compatible with a tumor of odontogenic origin. She was submitted to an extended resection, and the histology was consistent with a DGCT.DiscussionCentral DGCT affects mainly male patients between the fourth and sixth decades, with a predilection for the posterior portion of the jaws. The symptoms are unspecific, and a vast percentage of patients is asymptomatic. Radiographically a unilocular feature is commonly found, unlike this case. The recommended treatment is extended local resection due to its high recurrence rate.ConclusionDue to its rarity, knowledge of this entity is necessary for a better diagnostic and therapeutic guidance.     

Solid pseudopapillary neoplasm–Case report of a rare pancreatic tumor

IntroductionSolid pseudopapillary neoplasm (SPN) is a rare pancreatic tumor. Most patients are female within the second or third decade of life with only a small minority concerning children. Frequently described as low malignant potential tumors, surgical resection remains the main treatment.Presentation of caseThe authors present a case of a SPN diagnosed in a 17-year-old Caucasian girl with vomiting and abdominal pain localized to the right upper quadrant. CT scans and MRI scans showed the presence of a well-defined tumor arising from the pancreatic head with 14 cm of greater diameter. Pylorus-preserving pancreatoduodenectomy was performed and histopathology confirmed a SPN with complete resection and no evidence of malignancy.DiscussionSPN are usually found incidentally hence the importance of an accurate radiologic diagnosis. Symptoms may be present such as abdominal pain or vomiting due to compression, namely in large tumors. A surgical approach is usually indicated aiming complete resection, with tumor size not predicting resectability.ConclusionSolid pseudopapillary tumors of the pancreas are extremely rare and usually have an excellent long-term prognosis after surgical resection.     

Maxillomandibular brown tumor – a rare complication of chronic renal failure

We report a 17-year-old hemodialysis patient with a rapidly growing maxillary mass diagnosed as a brown tumor. Although successful control of the parathyroid hormone (PTH) levels was achieved by treatment with vitamin D pulse therapy, the lesion progressed, invaded the maxillary sinus, and caused severe eating and speech disabilities. No recurrence was observed following surgical excision. The differential diagnosis and considerations regarding the causes of the disease in a child, therapy options, and review of the literature are presented. Received: 26 January 1999 / Revised: 24 June 1999 / Accepted: 25 June 1999     

Primary carcinoid tumor of the filum terminale—a case report

Carcinoid tumors with a primary site in the central nervous system have not been reported in literature yet. We report here about a 41-year-old patient with recurrent and progressive low back pain and bilateral S1 radiculopathy on admission. The patient underwent hemi-laminectomies of the vertebral bodies L5 and S1 and an en bloc resection of the tumor. Postoperative histopathological examination resulted in a well-differentiated intrathecal neuroendocrine tumor (carcinoid) of the terminal filum. Postoperative staging showed no pathological abnormalities and no tumor recurrence after 6 months. Even though rare, carcinoids should be considered as differential diagnosis of tumors occurring in the CNS.     

Infiltrating giant cell tumor in a case of Paget’s disease of bone

Giant cell tumor (GCT) of the bone, also called osteoclastoma, is a rare complication of Paget’s bone disease. We report a patient from Southern Italy who developed a GCT infiltrating the neighboring tissues. The natural history and the therapeutic outcomes of this unique complication of Paget’s bone disease are presented.     

Reduction of tumorigenicity by an interferon-γ-gene-transduced tumor on another syngeneic tumor in a murine model

To evaluate the effect of interferon-γ-genetransduced cells, DS mice were inoculated into their footpads with syngeneic mammary adenocarcinoma SC42 admixed with interferon-γ producing mammary adenocarcinoma SC115Kγ, which had been established by an interferon-γ-gene transduction in another syngeneic mammary adenocarcinoma SC115 using retroviral vectors. These mice rejected both tumor cells and developed resistance to subsequent challenges with either SC115 or SC42 cells inoculated into their opposite posterior footpads. These results thus indicate that systemic immunological memory to each of the independent tumor cell lines developed in these mice. Although the SC42 cells admixed with irradiated SC115Kγ cells were rejected by these mice, the SC42 cells admixed with irradiated SC115neoR, in which the neo-gene had been transduced, were observed to proliferate. Tumor rejection was reversed by an in vivo administration of anti-interferon-γ antibody, thus suggesting that locally produced interferon-γ plays an important role in tumor elimination and immunological memory induction. In conclusion, interferon-γ-gene-transduced tumor cells are therefore considered to have a therapeutic potential for other types of malignant tumor cell lines.     

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.     

Rotationplasty as a salvage procedure in revision of tumor endoprosthesis of the distal femur – a report of two cases

We performed two rotationplasties (type A1 and A2) as salvage procedures in cases of custom- made endoprosthetic replacements of the distal femur and knee joint. The patients suffered from malignant bone and soft-tissue tumors of the knee, respectively. One of them developed chronic osteomyelitis around both stems of the prosthesis, and three local revisions turned out to be ineffective. The other patient, suffering from parossal osteosarcoma of the distal femur, developed a local recurrence following endoprosthetic reconstruction. The functional abilities following the operation of these two patients suggested that rotationplasty was a viable alternative to above-the-knee amputation in failed or severely complicated total arthroplasty of the knee with massive bone loss. Received: 22 April 1999     

Hyperparathyroidism–jaw tumor syndrome: a report of three large kindred

Background  Hyperparathyroidism–jaw tumor syndrome (HPT–JT) is a rare autosomal disease caused by inactivating germ-line mutations of HRPT2 gene, with subsequent loss of Parafibromin expression. It is characterized by familial HPT, ossifying jaw tumors, and other associated neoplasms. Methods  Clinical, histopathological, and genetic features of three large Italian unrelated HPT–JT kindred were assessed. Results  Three different germ-line HRPT2 inactivating mutations were identified. Seventeen affected members and six healthy mutation carriers were found. HPT was diagnosed in virtually all affected patients, at a median age of 36.3 years (range 11–71). In all cases, a single parathyroid involvement was found at surgery, although a metachronous multiglandular involvement causing recurrence after selective parathyroidectomy occurred in 17.6% of cases, after a mean disease-free interval of 13.7 years (range 5–27). Parathyroid carcinoma, atypical parathyroid adenoma, and jaw tumor occurred in one case; uterine involvement in 61.5% of women; other associated neoplasms were thyroid carcinoma (two cases) and renal and colon carcinoma (one case). Immunohistochemistry confirmed the loss of Parafibromin as the distinctive feature of the disease both in parathyroid and uterine tumors. Conclusions  HPT–JT has a frequent single-gland parathyroid involvement and a relatively increased risk of parathyroid carcinoma. The penetrance of the disease is high but incomplete. Regardless of the denomination of the syndrome, jaw tumors occur rarely, while uterine involvement is frequently present. Selective parathyroidectomy may be an effective strategy, but a prolonged follow-up is required because of the risk of recurrences and malignancies. A systematic investigation is also required because of associated malignancies. Best of Endocrine Surgery in Europe 2009     

Solid-pseudopapillary tumor of the pancreas – a rare and frequently misdiagnosed neoplasm

Introduction: We describe a young woman with an unusual pancreatic tumor. Findings and discussion: Intraoperatively, a smoothly demarcated and encapsulated tumor was exposed. It was large (5 cm×4 cm) and of solid consistency, with a small stalk attached to the uncinate process. The tumor was partially surrounded by the pancreatic head. The macroscopic appearance suggested a benign tumor. Frozen sections revealed a benign pancreatic tumor, most likely of endocrine nature. Based on these findings, tumor enucleation was performed. The patient recovered rapidly from the intervention and was discharged from hospital after 2 weeks. One year after surgical treatment, the patient is without recurrence. The final diagnosis of the tumor was a solid pseudo-papillary tumor. Received: 9 July 1998 Accepted: 14 September 1998     

Is laparoscopic resection the appropriate management of a jejunal gastrointestinal stromal tumor (GIST)? Report of a case

A 51-year-old female patient presented with iron deficiency anemia. Upper and lower gastrointestinal endoscopy were unremarkable. Computed tomography enteroclysis showed an ovoid 3×4-cm jejunal tumor with intraluminal protrusion and exophytic growth pattern, without lymphadenopathy or metastatic disease. Laparoscopic resection of the tumor was successfully carried out. Histologically, a mesenchymal tumor composed of spindle cells with an interlacing bundle pattern and high-mitotic activity greater than 10 mitoses/50 high-power fields were observed. The immunohistochemistry showed that the tumor was KIT (CD117)-, vimentin-, smooth muscle actin-, and S-100-positive, whereas it was CD34-negative. These findings were consistent with the features of a gastrointestinal stromal tumor. The patient had an uneventful postoperative course, and after 10 months of follow-up, she is well without any evidence of tumor recurrence.     

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.     

Is calcifying fibrous pseudotumor a late sclerosing stage of inflammatory myofibroblastic tumor?

Calcifying fibrous pseudotumor is a recently described distinctive lesion, characterized by the presence of abundant hyalinized collagen with psammomatous or dystrophic calcifications and a lymphoplasmacytic infiltrate. The cause and pathogenesis are unclear, but a possible relationship with other pseudotumors, like nodular fasciitis or inflammatory myofibroblastic tumor, has been proposed by some authors. However, cases with overlapping histologic features have not been reported. A 17-year-old girl with multiple peritoneal calcifying fibrous pseudotumors and inflammatory myofibroblastic tumors (inflammatory pseudotumors) is described. Some multinodular lesions showed calcifying fibrous pseudotumors next to inflammatory myofibroblastic tumors. Transitional stages between calcifying fibrous pseudotumor and inflammatory myofibroblastic tumor were also present. This case clearly illustrates a histogenetic relationship between calcifying fibrous pseudotumor and inflammatory myofibroblastic tumor, and it suggests that calcifying fibrous pseudotumor is a late sclerosing stage of inflammatory myofibroblastic tumor, at least in some cases.     

PPARγ2 functions as a tumor suppressor in a translational mouse model of human prostate cancer

Peroxisome proliferator-activated receptors y(PPARy)is a master regulator that controls energy metabolism and cell fate.PPARy2,a PPARy isoform,is highly express...