Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0392680 (shortness of breath)
 5,217 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An 18-year-old girl presented with severe chest pain and shortness of breath. She was found to have a large desmoid tumor filling the right chest cavity. The etiology of the desmoid tumor is unknown but is thought to be associated with an underlying genetic defect and local trauma. These tumors may be estrogen sensitive and are more frequent in females of reproductive age. The case demonstrates the difficulty in treating these tumors effectively.
 ...
PMID:Life-threatening desmoid tumor. 221 Dec 81

Intrathoracic desmoid tumors of the pleura are unusual tumors that are often clinically and histologically confused with localized fibrous tumor of the pleura or benign neurogenic tumors. We studied four cases of intrathoracic desmoid tumor of the pleura and reviewed the clinical, histopathologic, and immunohistochemical features of the four patients. Two men and two women, ranging in age from 16 to 66 years (mean, 44 yr) comprised the study group. Three patients presented with chest pain and one with shortness of breath. Two patients had a history of associated trauma in the area of the tumor. Three of the lesions were based in the parietal pleura and one in the visceral pleura. Treatment included complete resection (two cases), subtotal resection (one case), and subtotal resection followed by radiation therapy and complete resection (one case). The mean tumor size was 12.5 cm, and all of the tumors exhibited a bosselated, firm, white, cut surface. The histologic features of intrathoracic desmoid tumors were similar to those of desmoid tumors at more conventional sites. Infiltration of the adjacent fat and skeletal muscle was invariably present. The tumor cells were immunoreactive for vimentin, desmin, smooth muscle actin, and muscle-specific actin in three of the four cases and were negative for S-100 protein. Follow-up to date shows stable residual disease at 12 months (one case) and two patients with no evidence of disease at 12 and 96 months, respectively. Intrathoracic desmoid tumors often exhibit clinical and radiographic features similar to localized fibrous tumor of the pleura. They generally have histologic and behavioral characteristics identical to those of desmoid tumors at conventional sites. Like desmoid tumors elsewhere, complete resection with negative margins is vital to prevent local recurrence. Desmoid tumor should be considered in the differential of localized fibrous tumor of the pleura.
 ...
PMID:Desmoid tumors of the pleura: a clinicopathologic mimic of localized fibrous tumor. 995 Jan 56

We present a case report that describes the pathology, presentation, and management complexities of an unusual, destructive fibrosclerotic lesion of the laryngotracheal complex. An otherwise healthy 21-year-old man presented with a 1-year history of progressive shortness of breath and stridor. The initial examination revealed a 3-cm, grade III subglottic stenosis. Nodular fibrosis of the strap muscles, laryngotracheal cartilages, and trachea was evident. Biopsies revealed dense peritracheal desmoplastic reaction with focal erosion of cartilage. However, features diagnostic for relapsing polychondritis, desmoid tumor, or orbital pseudotumor were absent. The disease progressed to involve severe stenosis and thickening of the trachea and main stem bronchi. Surgical and medical management of this unusual fibrosclerotic lesion did not ameliorate the disease process, but a recent encouraging response to tamoxifen citrate has been observed with improvements in vocal fold motion and activity levels. Prognosis and management experience for this unknown pathologic entity are absent in the literature. In this case, diffuse disease progression occurred despite surgical and medical management, but has been halted by tamoxifen therapy. The prospect of a durable response and disease remission is unknown.
 ...
PMID:Unusual fibrosclerotic lesion of the laryngotracheal complex presenting as subglottic stenosis. 1924 60

Desmoid tumors are soft-tissue neoplasms arising from fascial or musculo-aponeurotic structures. Most reported thoracic desmoid tumors originate from the chest wall. However, intrathoracic desmoid tumors are rare. We present a case of a 35-year-old male patient complaining of mild shortness of breath. The patient was diagnosed to have a huge intrathoracic desmoid tumor, which was successfully resected.
 ...
PMID:Huge intrathoracic desmoid tumor. 1964 48