Fibrous dysplasia of the spine, costae and hemipelvis with sarcomatous transformation

We describe a patient with polyostotic fibrous dysplasia and secondary malignant fibrous histiocytoma in a spinal lesion.     

Comparative susceptibility of Glossina longipennis and G. brevipalpis to pathogenic species of Trypanosoma

A 56-year-old woman was admitted to our hospital with the chief complaint of left back pain. We detected left pleural effusion on chest X-ray and performed chest drainage. No malignant cells were detected in the effusion. Chest CT demonstrated a tumor shadow over the left diaphragm and left pleural effusion 6 months later. She underwent en bloc resection of the tumor and left diaphragm. Histological evaluation revealed malignant fibrous histiocytoma (MFH). Recurrent tumors were found in the abdomen 8 months after the operation, and she underwent resection of the abdominal recurrent tumors. Histological evaluation of the recurrent tumors also showed MFH. The patient died 28 months after the first operation.     

Primary malignant fibrous histiocytoma of the descending colon

We herein present the case of a 50-year-old woman with malignant fibrous histiocytoma arising from the descending colon and localizing in the colonic wall. Malignant fibrous histiocytoma of the large bowel is a very rare tumor. A total of 18 cases, including our case, have been reported in the world medical literature so far and we also reviewed these cases. In our case, combined adjuvant chemotherapy was administered after a complete resection had been performed. No clinical signs of local recurrence or distant metastasis were found at 7 years after the operation.     

A case of primary fibrous histiocytoma of the lung

Fibrous histiocytoma is non-epithelial malignant tumor mainly arising from soft tissue in extremities and body. Those derived from lung are rare. A 71-year-old female was admitted to our hospital because of an abnormal shadow on chest X-ray film. Preoperative diagnosis was not obtained by various examination. The tumor was located in right upper lobe (S2) and partial resection of right upper lobe was performed. Pathological diagnosis was fibrous histiocytoma of borderline malignancy. After the operation, adjuvant therapy was not done. The patient is doing well without recurrence and metastasis, during 4 years after the operation. However, careful follow-up should be necessary for long term.     

Congenital diaphragmatic hernia: an unsolved problem

Primary and metastatic malignant fibrous histiocytoma of the alimentary tract is uncommon, even though it is the most frequently diagnosed malignant soft tissue tumor in adults. In this report, we describe a case of malignant fibrous histiocytoma of the colon.     

Enhanced expression of catalytic subunit isoform PP1 gamma 1 of protein phosphatase type 1 in malignant fibrous histiocytoma

The expression of the three catalytic subunits of protein phosphatase (PP) type 1 and 2A, PP1 alpha, PP1 gamma 1, and PP2AC, was examined in malignant fibrous histiocytoma using immunohistochemical analysis. The percentage of cells stained positively with antiserum against PP1 catalytic subunit isoform PP1 gamma 1 was significantly higher in tumorous region than in non-tumorous region of malignant fibrous histiocytoma. Furthermore, tumorous region showed markedly high S-phase fraction in the cell cycle, as compared to non-tumorous region. These results suggest that PP1 gamma 1 is involved in the accelerated growth of tumor cells in malignant fibrous histiocytoma.     

Malignant fibrous histiocytoma metastases to the small intestine and colon presenting as an intussusception

A case of malignant fibrous histiocytoma metastases to the small intestine and colon presenting as an intussusception is described. Although malignant fibrous histiocytoma is the most common soft tissue sarcoma in late adult life, GI involvement has rarely been reported. The review of both our case and eight cases in the English-language literature suggests that GI involvement from malignant fibrous histiocytoma occurs most frequently in the small intestine (six of nine) and that two major clinical manifestations of GI involvement are GI bleeding (five of nine) from ulcerated tumors and intussusception (two of nine) led by polypoid tumors.     

Viral hepatitis A, E and their mix (A+E) in children

Atypical ("pseudosarcomatous"), cutaneous, fibrous histiocytoma is a rare connective tissue tumor arising on the trunk and limbs in young adults. Its histological diagnosis is difficult. We report the case of a 25-year-old woman who presented a nodule on her left leg. Two years after an incomplete excision, she developed a large local recurrence. Additional radiotherapy, after total reexcision was performed. This treatment was successful and no further recurrence occurred. Clinicopathological features of atypical ("pseudosarcomatous"), cutaneous, fibrous histiocytoma are reviewed. Differential diagnoses, including atypical fibroxanthoma, angiomatoid fibrous malignant histiocytoma and aneurysmal fibrous histiocytoma are discussed.     

The classification of pneumothorax]

We report a case of spontaneous rupture of malignant fibrous histiocytoma. A 50-year-old male with right flank pain was referred to our hospital. Computed tomography (CT) showed a heterogeneous space-occupying lesion on the upper pole of the right kidney. Selective right renal arteriography revealed a hypovascular mass. Preoperative clinical diagnosis was spontaneous rupture of renal cell carcinoma. Radical nephrectomy was performed. Histopathological diagnosis was malignant fibrous histiocytoma arising from the renal capsule.     

Neuroligand receptor heterogeneity among astroglia

The clinical and histopathological findings are described in a 39-year-old female patient with two different primary ophthalmic cancers, involving the adnexa and ocular globe of the same eye. The first primary tumor was a malignant fibrous histiocytoma of the left lower eyelid aggressively invading the nasogenian region, bulbar conjunctiva, episclera, and the cornea over a 36-year follow-up period. The second primary cancer was an unsuspected choroidal malignant melanoma unexpectedly found at histology. The possible correlations between these two malignancies are discussed.     

Isolated limb perfusion of an irradiated foot with tumor necrosis factor, interferon, and melphalan

A 57-year-old woman presented with the second recurrence of a high-grade malignant fibrous histiocytoma of the right foot, following previous local resection plus curative adjuvant radiotherapy. The first recurrence of the lesion was treated by isolated limb perfusion with cisplatin; the second recurrence was treated by isolated limb perfusion with tumor necrosis factor, interferon, and melphalan. The tumor and the area that had been irradiated showed a bluish color a few hours after tumor necrosis factor perfusion. Amputation of the right foot and leg below the knee had to be performed because of severe necrosis.     

Spirituality: an important aspect of emergency nursing

Fibrous histiocytoma has a variable, unpredictable behaviour and potential for malignancy. The treatment of choice is wide local excision, which, in the larynx, entails total laryngectomy. A case of fibrous histiocytoma of the larynx, treated successfully by radiotherapy, is presented. A recurrence was treated with cryosurgery and the patient is well at 10-year follow-up. The pathological characteristics of fibrous histiocytoma and the difficulties of classification are reviewed.     

Primary malignant fibrous histiocytoma of spleen

We report a case of primary malignant fibrous histiocytoma of spleen, which also emphasizes the difficulties in diagnosing such a rare condition.     

Single somatic ras gene point mutation in soft tissue malignant fibrous histiocytomas

The frequency of ras gene mutations in human soft tissue malignant fibrous histiocytomas within and around the hot spot codons (12, 13, and 61) of all ras genes, (H-ras-1, K-ras-2, and N-ras) was studied by nested polymerase chain reaction and direct DNA sequencing from archival formalin-fixed, paraffin-embedded tissue. Light microscopy and immunohistochemistry served to define malignant fibrous histiocytoma. All of the four differentiation subtypes (storiform-pleomorphic, inflammatory, myxoid, and giant cell) were investigated. Nine of thirty-two malignant fibrous histiocytomas (28%) contained ras gene point mutations. The highest incidence was found in the myxoid subtype (four of nine). H-ras-1 gene codon 12.2 was the only codon affected and contained in all mutated cases a GGC-->GTC exchange. Seven of the nine mutations were homozygous and probably affected more than 80% of the tumor DNA. The flanking regions of all hotspot codons did not contain any point mutation. The presence of a single and often homozygous point mutation of the H-ras-1 gene, especially in myxoid malignant fibrous histiocytoma could serve as a basis for further genomic discrimination of myxoid sarcomas.     

Extraperitoneal anterior approach to renal vessels in radical surgery of renal cancer

A 63-year-old female developed the chest wall tumor protruding into the right thoracic cavity. She consulted our hospital complaining of chest pain. 20 years before this episode, she had undergone tumor extirpation combined with the partial resection of adherent anterior chest wall and the diaphragm for large benign solitary fibrous tumor of the pleura. Because of the difficulty in making diagnosis by needle biopsy, operation (re-tumor extirpation combined with the chest wall) was done. Histological examination revealed that the tumor was malignant solitary fibrous tumor. We concluded that the tumor was the malignant recurrence of the benign solitary fibrous tumor resected 20 years before. Occasionally, benign solitary fibrous tumor of the pleura recurs and behaves more aggressively than primary, but such a long latent period as 20 years is quite uncommon. So we present the case and the review of the literature.     

LN-2 (CD74). A marker to distinguish atypical fibroxanthoma from malignant fibrous histiocytoma

BACKGROUND: In this study, the authors examined the expression of LN-2, an antigen expressed by B cells, macrophages, and Reed-Sternberg cells, in a variety of spindle cell lesions of the skin to determine whether LN-2 immunoreactivity can be used to differentiate among these tumors. For comparison, they examined CD34 antigen expression in these lesions, which has been shown to be a useful marker in differentiating dermatofibrosarcoma protuberans from dermatofibroma. METHODS: Immunocytochemistry with anti-LN-2 and anti-CD34 monoclonal antibodies on formalin fixed, paraffin embedded material was performed on 102 spindle cell lesions, including dermatofibroma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, malignant fibrous histiocytoma, leiomyoma, and neurofibroma. RESULTS: LN-2 immunoreactivity did not distinguish between dermatofibroma and dermatofibrosarcoma protuberans, both of which showed weak immunoreactivity. In marked contrast, 90% of cases of malignant fibrous histiocytoma showed strong staining for LN-2, whereas the vast majority (90%) of cases of atypical fibroxanthoma were negative or stained only weakly with anti-LN-2 antibodies. Of the two cases of atypical fibroxanthoma that stained strongly for LN-2, both lesions were > 2 cm in size and extended deep into the subcutaneous fat. CONCLUSIONS: Differential expression of the LN-2 antigen by atypical fibroxanthoma and malignant fibrous histiocytoma distinguishes these two lesions and suggests that acquisition of LN-2 positivity may be a marker of tumor progression.     

Value of imaging in the assessment of malignant fibrous histiocytoma of the soft tissues

Malignant fibrous histiocytoma (MFH) is a rare and potentially highly malignant sarcoma. The authors report 6 cases of MFH in various sites: two in the chest wall, one in the pelvis, two in the gluteal zones and one on the scalp. Ultrasonography and computed tomography were the main imaging methods used in the assessment of the structure and extension of the tumor. A poor prognosis was noted in four cases: death within a few months in the two thoracic sites, recurrence in the pelvic and scalp lesions, radical surgery allowed recovery in two cases. A review of the literature showed that MRI and CT are complementary in the initial staging and follow-up of these patients.     

Peroxisome proliferator-activated receptor gamma activators inhibit gene expression and migration in human vascular smooth muscle cells

Mesenchymal hamartomas of the liver are the second most common benign liver tumor of childhood. The experience with this tumor at Egleston's Children Hospital at Emory University from 1989 to 1994 is reviewed. Eight patients presented with abdominal distention or an upper abdominal mass. Six patients presented at a mean age of 8 months, and two patients presented at 17 and 23 years of age, respectively. Four patients displayed normal alpha-fetoprotein levels, whereas one patient had an elevated level. Liver function studies were normal in all patients. Abdominal ultrasonography and CT scans revealed a cystic, septated mass within the liver or on a pedicle in all patients. Five patients had simple excision of the tumor, and two had major hepatic resections. The cysts were multiloculated and lined with cuboidal bile duct epithelium surrounded by stroma containing proliferating bile ducts, blood vessels, and compressed liver tissue with no calcifications. In one patient, some pathologists favored the diagnosis of malignant myxoid fibrous histiocytoma because of similar-appearing stroma. Follow-up (mean, 35 months) revealed one symptomatic recurrence after initial resection was incomplete. There were no other recurrences and no malignant transformations. A septated, noncalcified, cystic hepatic mass in an infant with normal liver function studies and characteristic ultrasound or CT is likely a benign mesenchymal hamartoma that can be cured by total local excision.     

Testing basic visual functions in the evaluation of extrapyramidal side effects of antipsychotic agents

A female patient presenting with B-CLL and coincident eosinophilia-myalgia syndrome (EMS) after ingestion of L-tryptophan is described. The manifestations of EMS disappeared completely during treatment with cyclophosphamide/prednisone. and there was an intermittent clinical remission of CLL with absence of the monoclonal B-cell population. A few years later, the B-CLL relapsed, but without sign and symptoms of EMS. Whereas other eosinophilic syndromes such as eosinophilic fasciitis, panniculitis, or cellulitis Wells have been found to occur in relation to malignant underlying diseases, only a single patient with malignant fibrous histiocytoma following EMS has been described. There are no reports about an increased occurrence of B-CLL or other non-Hodgkin's lymphomas combined with or following EMS or related to L-tryptophan itself. The variant types of eosinophilic syndromes occurring due to malignant disorders, the differentiation from EMS, and the possible association between B-CLL and L-tryptophan-related EMS are discussed.     

Malignant fibrous histiocytoma of the hand: multimodal therapy and extensor tendon reconstruction

A patient with malignant fibrous histiocytoma of the dorsum of the hand, managed with irradiation and wide local excision, is presented. Biopsy technique, the rationale for preoperative irradiation, and the details of extensor tendon reconstruction are discussed. Tendon grafts, rather than transfers, were used to reconstruct the extensor tendon defects. Although this approach is simple, little is known regarding the healing of irradiated tendons or the use of dynamic splinting protocols in this setting. Our patient experienced no difficulty with either tendon healing or early dynamic splinting and achieved an acceptable range of motion.