Primary aneurysmal cyst of soft tissues (extraosseous aneurysmal cyst)

In this report we describe a soft-tissue aneurysmal cyst located in the left shoulder of a 20-year-old woman. Although the tumor initially was clinically considered a myositis ossificans, the microscopic features were identical to those found in classic aneurysmal bone cyst (ABC). Light microscopy showed a lesion composed of a peripheral band of mature trabecular bone surrounding a multicystic central area made up of anastomosing cavernous blood channels separated by fibrous septa that contained osteoid, mineralized myxochondroid material and osteoclastic giant cells. Whereas most bone tumors have an extraosseous counterpart, ABC has been found outside the bone only once, in an artery, but never in soft tissues. Moreover, major reviews state that aneurysmal bone cyst occurs exclusively within bones. This unique case is a very rare occurrence of one of these lesions in the soft tissues.

Parathormone secretion in peritoneal dialysis patients with adynamic bone disease.

The prevalence of low-turnover lesions in patients undergoing peritoneal dialysis (PD) is high. Our aims are to evaluate the prevalence of adynamic bone disease (ABD) in PD patients, analyze risk factors, and define the association of serum parathyroid hormone (PTH) levels measured under different plasma calcium concentrations with this lesion. Fifty-seven patients were studied by bone biopsy (BB). ABD was found in 63.2%, and 36.8% showed high-turnover bone disease (HTBD). Patients with HTBD had a lower prevalence of diabetes, younger age, lower accumulated oral calcium salt intake, and greater calcitriol doses, serum osteocalcin level, and ultrafiltration than patients with ABD. Both mean baseline PTH levels from the previous year and PTH level at time of BB were greater in patients with HTBD than those with ABD (357 +/- 267 pg/mL versus 89 +/- 67 pg/mL; 390 +/- 337 pg/mL versus 88 +/- 78 pg/mL, respectively; P < 0.05). However, the magnitude of the increase from baseline serum PTH levels in response to hypocalcemia was greater in patients with ABD than in those with HTBD (166.4% +/- 134% versus 83.5% +/- 73.6%; P < 0.05). We found that PTH levels less than 150 pg/mL in patients with ABD showed a sensitivity of 91. 6%, specificity of 95.2%, and positive predictive value (PPV) of 97%. In the HTBD group, PTH levels greater than 450 pg/mL had a specificity and PPV of 100%. Our data confirm that ABD is the most prevalent lesion in PD patients, and PTH secretion capacity is maintained in these patients. The definitive diagnosis and management strategies for many patients requires a BB, especially when HTBD is unlikely.

Cultures of human osteoblastic cells from dialysis patients: influence of bone turnover rate on in vitro selection of interleukin-6 and osteoblastic cell makers.

The factors contributing to renal osteodystrophy are still incompletely characterized. A variety of cytokines and growth factors appear to have ill-defined roles in this disease. Our aim is to compare osteoblastic cell growth and different osteoblastic markers in vitro with histomorphometric bone parameters and some serum bone-turnover markers in vivo in dialysis patients with either high- (HTBD) or low-turnover (LTBD) bone disease. Six patients were diagnosed to have LTBD, and another five patients, HTBD. Intact parathyroid hormone (PTH) and osteocalcin (OC) levels in serum were greater in patients with HTBD than in those with LTBD. Osteoblastic cells isolated from iliac crest biopsy specimens were grown in culture medium for different times up to 13 days. Osteoblastic cell growth (cell number and area under the cell growth curve) was greater in patients with HTBD than in those with LTBD. Static and dynamic bone formation parameters correlated with serum PTH levels. No correlation was found between PTH and osteoblastic cell proliferation. OC, C-terminal type I procollagen, and alkaline phosphatase osteoblastic secretion in vitro were similar in the HTBD and LTBD groups. However, interleukin-6 (IL-6) secretion was greater in cells isolated from patients with LTBD. Our results indicate that osteoblastic cell growth and osteoblastic IL-6 secretion are related to bone turnover in patients with osteodystrophy. Our findings support the hypothesis that factors other than PTH level might have an important role in affecting osteoblastic function in renal osteodystrophy.

Primary aneurysmal cyst of soft tissue. Report of a case with ultrastructural and MRI studies.

We report a case of primary aneurysmal cyst of soft tissues in a 57-year-old woman presenting with a painful mass in her left arm. Conventional radiography showed a radiolucent soft tissue mass surrounded by a ring of bone. MRI displayed an unusual, ill-defined soft tissue lesion that was not connected to the nearby humerus and appeared to be an aggressive tumour. Microscopically, the mass consisted of multiple anastomosing cavernous channels surrounded by a peripheral band of mature trabecular bone. These bloody channels were separated by fibrous septa containing fibroblasts, histiocytes and multinucleated giant cells, as well as fibromyxochondroid material. Some of these giant cells lined the septa and partially occupied the lumen of the channels. Ultrastructurally, the features observed in this tumour were similar to those described in aneurysmal bone cyst; the giant cells lining the septa were an additional observation. Whereas most bone tumours have a well-known extraosseous counterpart, this unique lesion is not well recognized by surgical pathologists and the few published cases have been reported under different names. Gross, microscopic, radiological and ultrastructural findings are presented to familiarize pathologists with this underdiagnosed condition.

Calcified leiomyoma of deep soft tissue. Report of a case in childhood.

This report illustrates a calcified leiomyoma of deep soft tissue in the left leg of a 6-year-old boy. The tumour was composed of spindle cells arranged in interlacing bundles, between which were multiple small and large areas of calcification. Tumour cells were positive for vimentin, desmin and smooth muscle actin. Ultrastructurally, the cells showed numerous pinocytotic vesicles and bundles of intracytoplasmic filaments with smooth muscle dense bodies. Only four calcified leiomyomas have been previously reported in the deep soft tissues of limbs. Here we report a new case and suggest a new pathogenetic scheme involving alkaline phosphatase in the origin of these calcifications.

Primary epithelioid leiomyosarcoma of bone. Case report and literature review.

We describe an epithelioid leiomyosarcoma of bone located in the right knee of a 51-year-old woman. Plain radiograph and CT scan revealed a poorly defined lytic and destructive mass in the upper metaepiphyseal right tibia which involved surrounding soft tissues. The lesion was composed of proliferating monotonous round cells with a high mitotic activity with scanty intersecting spindle cell fascicles. Immunohistochemistry of both areas demonstrated a strong positivity for actin (HHF-35 and alpha-SMA) and vimentin, and negative reactions for desmin, keratin (AE1 AE3), epithelial membrane antigen, S-100 protein, factor VIII-related antigen, CD 31 and CD 34. Ultrastructural study confirmed a diagnosis of leiomyosarcoma. This is the first detailed description of the microscopic and radiological features of primary epithelioid leiomyosarcoma of bone.

Ewing's tumor in brothers. An unusual observation.

Ewing's sarcoma in two brothers is described. This has not been previously reported in the literature. Hypotheses which might explain this association are discussed. Genetic factors predisposing to the development of neoplasia are a very attractive possibility, although viral factors and chance association cannot be excluded.

Osteoblastoma and osteoid osteoma. Clinical and morphological features of 162 cases.

A study of 46 osteoblastomas and 91 osteoid osteomas, selected from a total of 162 cases proceeding from 15 Spanish Hospitals was done to establish epidemiological data on clinico-pathological parameters and to compare them with the findings of other authors. A comparative study between both tumors is done and emphasis is put on the discussion of aggressive osteoblastoma.

Ewing's sarcoma of the mandible: radiologic features with emphasis on magnetic resonance appearance.

Ewing's sarcoma is an uncommon malignancy that usually occurs in children. A case of Ewing's sarcoma of the mandible is presented, and the radiologic appearance is described, with special consideration given to the magnetic resonance imaging features.

[Hormonal multiplicity of an apudoma of the lung and pancreas. Characterization of the different peptides in the tumoral extracts (author's transl)]. / Multiplicidad hormonal en apudoma de pulmón y páncreas. Caracterización de los diferentes péptidos en los extractos tumorales.

The capacity which the cells of some tumors have of synthesizing, storing, and releasing hormonal polypetides constitutes the basic characteristic of the neoplasms of the APUD system. On many occasions these polypeptides are released as hormonal precursors of high molecular weight, with a minimal biological action in comparison with the real hormone (big ACTH, big gastrin, etc.), and they have no clinical expressivity. On other occasions they reproduce, however, the clinical syndrome of the hormone released in excess. The production of multiple hormones by a single tumor is not a common event. Here we present the case of a patient with an oat-cell carcinoma of the lung and a carcinoma of the pancreas, both histopathologically primitive. In this patient a syndrome of inadequate secretion of antidiuretic hormone was detected. By means of radioimmunoassay techniques, the existence of antidiuretic hormone, ACTH with a predominance of the components of high molecular weight (big ACTH and beta-LPH) and MSH was demonstrated in the tumoral extracts from the lung, pancreas, and from a mediastinal metastatic lymph node. While the concentrations of ACTH were much greater in the lung than in the pancreas, the opposite occurred for the antidiuretic hormone. The synthesis of MSH by the hypophyseal gland or by tumors is not at present recognized, but rather is considered as a degradation product during the process of extraction. The APUD system makes up the morphologic substrate of the syndromes of familiar multiple endocrine adenomatosis. The present case could represent a variant of sporadic multiple endocrine neoplasms which would have the same anatomical basis.

[Hepatic actinomycosis]. / Actinomicosis hepática.

We present a case of primary liver actinomycosis in a patient who was operated on for a duodenal ulcus seven years before. A gastrectomy was performed. His gallbladder was also removed two years before. After the surgical treatment, followed by antibiotic and chemotherapeutic treatment, with the result of a pretended success, the patient developed a liver pyogenic abscess and portal hypertension. Authors emphasise the seriousness of the illness as well as its rarity even with an accurate treatment.

Diaphyseal chondroblastoma in a long bone: first report.

Chondroblastoma is a rare benign bone tumor typically located in the epiphysis. We describe the first case of chondroblastoma arising in the diaphysis of a long bone. The patient was a 13-year-old girl who presented with pain over her right thigh. Radiographs showed a lytic lesion in the diaphysis of her right femur. A core biopsy and a subsequent surgical resection were performed.

[Hypertensive pulmonary vascular disease in children. Post-mortem study of 34 cases (author's transl)]. / Enfermedad vascular pulmonar hipertensiva en niños. Estudio autópsico de 34 casos.

Report of a post-mortem study in 34 infants having presented pulmonary hypertension. Ages were between 16 days and three years old. One of the patients showed pulmonary lesion because of plexogenic arteriopathy of unknown etiology. The other 33 cases had congenital heart malformations. Macroscopic data as heart weight, thickness of the wall of the right ventricle and circumference of the pulmonary artery trunk were analized. Lesions developed in the pulmonary artery and its branches as well as those of the pulmonary veins are described. Different patterns of the hypertensive vascular disease are refered, emphasizing their evolution with the age of the patients. Some of the cases are particularly analized because of clinical, physiopathological or morphological peculiarities.

Osteogenic sarcoma of the maxilla and mandible.

Osteogenic sarcoma of the maxilla and mandible is a peculiar entity within the context of the malignant bone tumors of other sites. In our series of nine cases the mean age at appearance was 34.1 years; 44.5% of the tumors affected the maxilla, and 55.5% affected the mandible. Most of the tumors were principally rapidly growing and painless or minimally painful masses. Histopathologically, the fibroblastic type was the most frequent, with prevalence of Broders' Grades III and IV. Radical surgery was the only therapeutic procedure in 87.5% of the series, producing a survival of 85%. Overall survival for all the therapeutic categories was 75%, with a mean disease-free interval of 8 years. In the remaining cases, local recurrence and intracranial invasion were the main causes of death.

Primary giant cell tumor of soft tissues similar to bone giant cell tumor: A case report and literature review.

In this report we describe a primary giant cell tumor (GCT) of soft tissues located in the left dorsal wrist of a 52-year-old man. Plain radiographs did not reveal any lesion in his carpal or hand bones. Although the tumor was clinically considered a ganglion initially, the microscopic features were identical to those found in classic GCT of bone. Light microscopy showed a lesion composed of a homogeneously mixed proliferation of spindle and polygonal mononucleated stromal cells and evenly distributed multinucleated, osteoclast-like giant cells. Whereas most bone tumors have an extraosseous counterpart, only 13 cases of GCT in soft tissues had been published until 1998. Moreover, 64 new cases have been reported in three series. Nevertheless, most major reviews and textbooks do not consider this tumor as a specific entity and regard it as a low grade variant of malignant GCT of soft tissue. We describe the clinical, histologic, and immunohistochemical features of this rare benign neoplasm emphasizing the differential diagnosis with its malignant soft tissue counterpart, an ominous tumor.

Benign metastasizing giant-cell tumor of the hand. Report of a case and review of the literature.

Benign metastasizing giant-cell tumor of the hands is an extremely infrequent neoplasm. A 37-year-old man with one of these neoplasms in the second phalanx of his fifth left finger was examined. Microscopically, the tumor was a conventional Grade 1 giant-cell tumor that metastasized to the lung. Although this tumor is most infrequent, its metastatic potential indicates inclusion of benign metastasizing giant-cell tumor in the differential diagnosis with other giant-cell neoplasms of the hands.