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J Fernando Val-Bernal

Publications and source records attributed to J Fernando Val-Bernal.

At least 37 records · Page 2Linked to original sources

Incidental in vivo detection of an epithelioid hemangioendothelioma of the mitral valve.

Valvular epithelioid hemangioendotheliomas (EHE) are exceptional. To the authors' knowledge only four cases have been reported. Herein is described an EHE incidentally detected in the mitral valve of a 69-year-old woman with chronic rheumatic valvular heart disease. The 0.4 cm lesion was situated in the anterior leaflet and was discovered in the pathological study after mitral valve replacement. The patient was alive and well 6 months after operation. Review of the literature including the present report, revealed that the mean age was 60.2 years (range, 49-69 years). Symptomatic patients had clinical features of valvular disease or embolism. Each of the four cardiac valves can be affected. Mean tumor size was 2.4 cm (range, 0.4-8 cm). In 40% of cases the EHE was an incidental finding at autopsy or in a removed valve. In two cases the involved cusp was affected by chronic rheumatic disease. In conclusion, EHE is a rare lesion that can be an incidental finding and it should be considered in the differential diagnosis of valve tumors. Although EHE can present a histologically benign appearance, the correct pathological diagnosis is clinically important because the lesion can be potentially malignant. Regular follow up is suggested due to this potential.

Aged↗

Nonspecific (idiopathic) granulomatous prostatitis associated with low-grade prostatic adenocarcinoma.

Nonspecific granulomatous prostatitis (NSGP) is uncommon and may simulate carcinoma both clinically and microscopically. Concurrent NSGP and prostatic adenocarcinoma is rare. To our knowledge this association has been documented once and it was only rarely mentioned in two large series of NSGP. We describe a 67-year-old man who presented with a history of prostatism of 1 month's duration. Suprapubic prostatectomy revealed NSGP associated with nodular hyperplasia and low-grade prostatic adenocarcinoma. The pathologist should be aware of the rare association of NSGP and prostatic adenocarcinoma. Wide sampling of the prostatectomy specimens with NSGP is mandatory to exclude an occult prostatic adenocarcinoma.

Adenocarcinoma↗

Purpuric generalized lichen nitidus: an unusual eruption simulating pigmented purpuric dermatosis.

BACKGROUND: Generalized haemorrhagic lichen nitidus is rare. To our knowledge, this form of presentation has only been reported once. OBJECTIVE: To describe a new case of generalized haemorrhagic lichen nitidus simulating a pigmented purpuric dermatosis. METHODS AND RESULTS: We document a 24-year-old man who presented with an 8-month history of a progressive non-pruritic, red-brown papular eruption on the dorsa of the feet, ankles and distal third of the legs. A diagnosis of Schamberg's progressive pigmentary dermatosis was made, and no treatment was prescribed. Two months later, the lesions had extended to the abdomen, groins, forearms, elbows and wrists. Biopsy of the skin of the right foot revealed lesions typical of lichen nitidus with subepidermal extravasation of red cells and capillary wall hyalinization. Macrophages and T lymphocytes were abundant in the infiltrate. CONCLUSION: Purpuric generalized lichen nitidus should be included in the differential diagnosis of pigmented purpuric dermatoses.

Administration, Topical↗

Primary purely intradermal pleomorphic liposarcoma.

INTRODUCTION: Pleomorphic liposarcoma (PLPS) is a high-grade pleomorphic sarcoma, containing multivacuolated lipoblasts, which usually develops during late adult life. It usually occurs in the deep soft tissues and uncommonly arises in the subcutis, the dermis representing an exceedingly rare site of occurrence. METHODS: We describe a case of PLPS arising in the dorsal aspect of the nose of a 75-year-old woman. Preoperative duration was 9 months. RESULTS: The lesion was intradermal well-circumscribed, dome-shaped, measuring 1.2 cm. It fulfilled the histologic criteria for inclusion in the PLPS category. The tumor cells focally expressed calretinin. A review of the literature yielded four cases documented, to which we add the present report. All the patients were adults with a mean age of 67 (range 39-95) years, and three of five cases arose on the scalp. Local recurrence occurred in one patient, but no distant metastases or disease-related deaths were observed. CONCLUSION: PLPS very rarely arises in the dermis. In spite of high-grade morphology, the intradermal tumor shows a relatively favorable prognosis. Diagnostic consideration includes pleomorphic lipoma, clear cell atypical fibroxanthoma, balloon cell melanoma, and metastatic clear cell carcinoma of renal origin. Recognition of this distinctive and rare type of liposarcoma is essential to avoid under- or misdiagnosis and inappropriate treatment.

Aged↗

Anticipated diagnosis of left atrial myxoma following histological investigation of limb embolectomy specimens: a report of two cases.

Nonfamilial myxoma occurs as a random event. The tumor is rare and can mimic other diseases. Cardiac myxomas should always be considered as a source of embolization, which need meticulous investigation and prompt indication of surgical resection. Tumors with a villous surface are prone to embolize. We report two cases of cardiac myxoma presenting as acute ischemia of one or two limbs due to embolic phenomena. The patients were females aged 55 and 37 years. Histological study of emboli taken from obstructed limb arteries in the two patients showed a picture indicating systemic embolization of a cardiac myxoma. The embolic tissue fragments showed the gross characteristics (i.e. villous surface) of the cardiac tumor. Further echocardiography and surgical removal confirmed the cardiac myxoma. Immunohistochemical study of embolectomy material disclosed strong reactivity of the tumor cells for calretinin. The histological examination of the embolectomy material can anticipate the cardiac lesion and its gross features. Calretinin is a useful marker in the differential diagnosis of cardiac myxoma with a myxoid thrombus. The necessity of histological examination of the embolectomy material is stressed.

Adult↗

Clinicopathologic study and DNA analysis of 37 cardiac myxomas: a 28-year experience.

PURPOSE: This study was performed to identify morphologic features of cardiac myxomas related to embolism and to provide a better understanding of the biology of these tumors, mainly in relation to their interleukin (IL)-6 expression and DNA content. PATIENTS AND METHODS: A total of 37 cardiac myxomas were reviewed retrospectively in a clinicopathologic study that included the correlation of echocardiographic and pathologic findings in 25 cases, together with immunohistochemical evaluation of IL-6 expression and flow cytometric DNA analysis of 35 tumors. RESULTS: There were 24 female patients and 13 male patients. The mean (+/- SD) age was 52 +/- 15 years. Fifty-four percent of patients presented with dyspnea, 51% presented with increased erythrocyte sedimentation rate (ESR), and 27% presented with embolic episodes, which were significantly associated with villous surface tumors. Atrial fibrillation was registered in 19% of patients and was significantly associated with large left atrial myxomas. Echocardiography proved to be a reliable method for preoperative diagnosis and for predicting tumor size and morphology. There was no perioperative mortality or long-term recurrences. The frequency of early surgical complications was associated with a longer mean ischemic time. Seventeen percent of tumors had abnormal DNA content, and 74% of tumors showed immunohistochemical expression of IL-6. Neither of these factors showed a significant association with embolism or constitutional illness. CONCLUSIONS: Villous surface myxomas are related to embolism, and large left atrial tumors are related to atrial fibrillation. Echocardiography is a reliable method with which to predict tumor size and morphology. Myxoma cells usually express IL-6, and some tumors have abnormal cellular DNA content. Surgical excision of the tumor is a safe and effective treatment.

Adolescent↗

[Hormone expression and opioid receptors in fetal and adult lung].

OBJECTIVES: To describe the cellular distribution and level of expression of certain hormones and opioid receptors during fetal development and in the lung of the healthy adult. METHOD: We sampled lung tissue from fetuses at three stages of development (pseudoglandular, canalicular and saccular) (3 samples per stage), from newborn infants (3), from 10-month-old infants (2) and from adults (3) who had died without lung disease. After specific immunohistochemical staining for hormones (calcitonin, parathormone, serotonin and adrenocorticotropic hormone - ACTH) and opioid receptors, we assessed the percentage of positive cells for each cell type in each sample. RESULTS: Serotonin is the first to appear (pseudoglandular stage in isolated neuroendocrine cells) and it disappears later. Calcitonin appears in the canalicular stage in neuroendocrine and lung cells. Expression is at its peak at birth and is less in the adult lung. We found no ACTH or parathormone production. Opioid receptors appear in the canalicular stage and peak at birth. In adult lung, bronchiolar muscle and mesothelial cells, only delta-type opioid receptors are present. CONCLUSIONS: Pulmonary hormone secretion is significant during fetal development and peaks at birth. Calcitonin is the main hormone produced in the fetal lung. Opioid receptors are present during fetal development in various types of cells and peak at birth. An understanding of the expression of active substances could have therapeutic relevance in certain conditions, such as bronchial asthma or respiratory distress syndrome in the child.

Adrenocorticotropic Hormone↗

Cutaneous lipomatous neurofibroma.

We report a cutaneous lipomatous neurofibroma on the skin of the left-side parietal area of approximately 9 months' duration in a 67-year-old woman. The regular distribution of adipose tissue throughout the lesion suggested that fat was an integral part of the tumor, not a metaplastic or degenerative process. To our knowledge, this type of lesion has not been documented. The main differential diagnosis embraces neurocristic cutaneous hamartoma, lipoma and its variants, cutaneous meningioma, and neural nevus with fat replacement. We propose that lipomatous neurofibroma of the skin is caused by aberrant development of adipose tissue in a neurofibroma. The lesion originated as pluripotential neural crest cells after migration. This acquired lesion could arise from local stem cells. The old suggestion that neuroectoderm is capable of mesenchymal differentiation may be relevant to the histogenesis of this neoplasm.

Adipose Tissue↗

Fibroepithelioma of pinkus with tumor giant cells.

A case of fibroepithelioma of Pinkus with pleomorphic epithelial giant cells is reported. The lesion was an ovoid polypoid nodule measuring 4 mm x 3 mm x 2 mm and was located close to the right axilla in an 86-year-old woman. The immunohistochemical features of the epithelial giant cells indicate that most of these cells are not cycling. We suggest that these cellular changes may represent a senescent event. Giant cells showed a mean nuclear major diameter more than twice that of small cells. Flow cytometric study of the tumor showed a hypodiploid DNA content and an intermediate grade S-phase fraction of the aneuploid component. To the best of our knowledge, a pleomorphic variant of Pinkus fibroepithelioma has not been reported to date. In fibroepithelioma of Pinkus, the correct diagnosis depends primarily on the architectural pattern of the tumor rather than on its cytologic features.

Aged↗

Concurrent tubulovillous adenoma and transitional cell carcinoma associated with diffuse gastric and intestinal metaplasia of the defunctioned ureter.

Villous adenoma is a common lesion of the gastrointestinal tract, but it is rare in the ureter. Thus, as far as we know, only one case limited to this location has been described. Intestinal metaplasia of the urothelium is not rare. However, only one case of gastric metaplasia with pseudopyloric glands has been described in the literature. We here report in detail on a tubulovillous adenoma of the ureter associated with diffuse gastric and intestinal metaplasia and a concurrent primary, solid, high grade transitional cell carcinoma, with extensive clear cell change, in a 56-year-old male patient. He had undergone a left nephrectomy for renal tuberculosis twenty years earlier, and the lesions developed in the ureteric stump. To the best of our knowledge, such a combination of lesions has not been reported previously either in the ureter or in the rest of the urinary tract. The coexistence of diverse lesions in our case might represent the pluripotentiality of the urothelium in association with chronic inflammation and neoplastic induction. The present report also emphasizes the metaplastic and malignant potential of a defunctioned urothelial structure. This case is of particular interest, because these coexistent lesions arose simultaneously with an anatomically separate adenocarcinoma of the rectum (Dukes' B). The patient died 76 days after admission. The dismal prognosis of our case was determined by the advanced anatomical stage and the histological high grade of the transitional cell carcinoma of the ureter.

Adenoma, Villous↗

Spindle cell rhabdomyosarcoma in adults. A case report and literature review.

Spindle cell rhabdomyosarcoma, a recently described variant of embryonal rhabdomyosarcoma in children, carries a favorable prognosis when compared with other types of rhabdomyosarcoma. This tumor is rare in adults, and only four cases have been documented previously. The clinicopathological study of such a case occurring in the retroperitoneal space of a 53-year-old man is herein reported. The patient died of uncontrolled local recurrence and hepatic metastases seven months after diagnosis. Based on the analysis of the data of the five cases reported, including the present one, it can be stated that spindle cell rhabdomyosarcoma in adults is not associated with the favorable outcome observed in the pediatric population.

Biomarkers, Tumor↗

Symptomatic intracavitary (noninvasive) cardiac metastasis from low grade endometrial stromal sarcoma of the uterus.

We report a 49-year-old woman who was operated upon 33 years ago for uterine endometrial stromal sarcoma of low-grade malignancy. The patient showed obstruction of the right ventricular outflow tract. An echocardiogram and MRI showed that a mass emanating from the inferior vena cava filled the right atrium, the right ventricle, and part of the pulmonary artery. The entire intracavitary neoplasm was successfully removed and diagnosed as low-grade endometrial stromal sarcoma. To our knowledge, this is the second report of a low-grade endometrial stromal sarcoma reaching the heart via the inferior vena cava. In this case an intracavitary metastasis may be a feature indicating the progression of the endometrial stromal sarcoma. Therefore, this neoplasm should be included in the differential diagnosis of cardiac intracavitary neoplasms. Patients affected by this tumor may benefit from early identification and resection of the obstructing neoplasm.

Endometrial Neoplasms↗

Primary amyloid tumor (amyloidoma) of the jejunum with spheroid type of amyloid.

The present report documents an incidentally discovered, solitary amyloidoma of the jejunum in a 70 year old man with no predisposing disorder or other sites of deposition of amyloid, as was demonstrated at autopsy. Amyloid tumors of the intestine have been reported only rarely. In those reports the deposits were conventional in type, forming large masses of acellular, eosinophilic, homogeneous material. In the case described here the deposits were in the form of corpora amylacea-like structures, or spheroids. To the best of our knowledge, the corpora amylacea-like structures, or spheroid amyloid, have not been previously described in bowel amyloidoma. This rare form of amyloid deposition should be recognized so as to prevent misdiagnosis.

Aged↗

Amyloidosis of the rectum mimicking collagenous colitis.

Gastrointestinal involvement in cases of systemic amyloidosis is very common. In the colorectal mucosa, amyloid deposition is ordinarily seen around vessels or diffusely in the lamina propria. We report two cases in men aged 69 and 29 years, whose rectal biopsies revealed prominent subepithelial amyloid deposits mimicking collagenous colitis. The amyloid deposits were composed of AA protein. A review of the literature has yielded only one previously reported case. Pathologists should be aware of this deposition pattern of amyloid to prevent misdiagnosis. Our cases underline the importance of the routine use of histochemical stains for amyloid in all cases of colorectal biopsies showing histologic changes suggestive of collagenous colitis.

Adult↗