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Biomedical subjects

I Janecka

Publications and source records attributed to I Janecka.

12 recordsLinked to original sources

Spindle cell rhabdomyosarcoma (so-called) in adults: report of two cases with emphasis on differential diagnosis.

Spindle cell rhabdomyosarcoma (RMS) is a recently described variant of embryonal RMS that carries a relatively favorable prognosis when compared with other forms of RMS. To date, spindle cell RMS has been described only in children. The authors have identified two unusual cases occurring in adults using the following criteria: tumors composed mainly of fascicular, relatively monomorphic spindle-shaped cells that show unequivocal immunohistochemical and ultrastructural evidence of myogenic differentiation. The tumors were identified in a 38-year-old woman and a 56-year-old man, arising in the cheek and left hemidiaphragm, respectively. Both were treated with surgical resection and chemotherapy. The first patient died of uncontrolled local recurrence of her tumor at 27 months after diagnosis, and the second died of metastatic disease at 13 months follow-up. The tumors were composed mainly of fascicles of spindle cells with palely eosinophilic cytoplasm admixed diffusely with sparse polygonal, rounded, or strap-shaped rhabdomyoblasts with brightly eosinophilic cytoplasm and with cross-striations in the first case only. Immunostaining for muscle-related antigens showed staining for smooth-muscle actin (focal), pan-actin HHF-35, desmin, fast myosin, myoglobin, and MyoD1. Both cases were negative for S-100 protein. On electron microscopy, both cases showed neoplastic rhabdomyoblasts with clear-cut sarcomeric differentiation in many of the tumor cells. Spindle cell RMS poses special problems in differential diagnosis when arising in adults and should be distinguished from leiomyosarcoma, malignant peripheral nerve sheath tumor with heterologous rhabdomyoblastic differentiation (malignant Triton tumor), and fibrosarcoma. In view of the good prognosis afforded children with spindle cell RMS and in light of the chemoresponsive behavior of RMS in general, we feel that it is important to identify tumors that meet the criteria for spindle cell RMS occurring in the adult population. However, based on these two cases, it is possible that spindle cell RMS occurring in adults may not be associated with such a favorable outcome.

Actins↗

Clonal chromosomal aberrations in a leiomyosarcoma of the sinonasal tract.

We report the first cytogenetic analysis of a leiomyosarcoma of the sinonasal tract, a rare neoplasm. Karyotypic analysis showed near-triploid and near-tetraploid modal chromosome numbers with extensive structural and numerical aberrations. Three consistent structural changes, including i(6p), der(10)ins(10;1)(q26;q23q44), and der(12)t(1;12)(q11;q24) were observed in most cells. A der(11)t(11;?)(p15;?) was observed in 14 of 20 cells. Clonal structural rearrangements, including i(1q), del(2)(q37), der(3)t(3;?)(p25;?), del(4)(q31), del(7)(q32), der(12)t(12;?)(p12;?), der(15), del(21)(q22), and der(X) were each observed in a few cells. Numerical changes, including trisomies for chromosomes 2-5, 7, 9, 11, 15, 17, 18, and 20 and monosomies 10 and 12 were observed. Comparison of our findings to those of leiomyosarcomas at different sites showed trisomies 7 and 20 and rearrangements of 11p12-p15 and 21q22.

Adult↗

Surgical resection followed by brachytherapy for malignancies involving the base of the skull.

Between January 1990 and July 1991, 14 patients were referred to the University of Pittsburgh for cranial base surgery following diagnosis of end-stage primary or recurrent neoplasms of the base of the skull. The treatment approach consisted of resection of the tumor along with placement of afterloading nylon catheters for iridium-192 implantation in areas where remaining tumor was believed to be present. A median dose of 3600 cGy (range, 2500 to 6000) was delivered to the tumor bed 1 to 14 days following surgery. with a median follow-up of 9 months, three patients remain alive without disease at 18, 19 and 36 months, and two are alive with disease at 5 and 31 months. Six patients died of disease at 3, 3, 3, 7, 16, and 17 months and three patients died of intercurrent disease at 1, 5, and 11 months. Cranial base surgery followed by brachytherapy appears to be a viable alternative in patients who otherwise have exhausted other methods of treatment. Careful attention to preoperative evaluation, surgery, and postoperative recovery should be given in order to prevent major complications.

Journal Article↗

Three-dimensional computed tomography.

Three-dimensional images were generated by reformating data from an axial image set. Separate contours were generated for bone and for tumor. The contour representing the tumor was generated by tracing the tumor using a cursor on the CRT. The contours showed the relationship of the tumor to the skull base. Three-dimensional imaging by this method is more of a communication tool than a diagnostic tool. The information on such images can be derived from close analysis of the axial image set. Indeed, determination of the position of the tumor relative to very precise structures requires analysis of the original images. The three-dimensional imaging does orient the observer and give an overall view of the position of the tumor. Because the contour of the tumor is traced, the final image represents the opinion of the radiologist. Various technical considerations and artifacts are discussed.

Journal Article↗

Angiofibroma: a flow cytometric evaluation of 31 cases.

Angiofibromas are histologically benign but locally aggressive tumors that often extend beyond the nasopharynx to involve contiguous structures. In addition, attempts to remove the tumors surgically are often frustrated by local recurrences. We studied 31 angiofibromas by flow cytometry to determine if tumor ploidy could be used to identify those tumors that might behave more aggressively. The patients, all males between 5 and 23 years of age, were divided into two groups. The 21 patients with tumors confined to the nasopharynx or paranasal sinuses were regarded as having local disease, the ten with tumors involving the infratemporal fossa, orbit, cheek, or intracranial structures were regarded as having extensive disease. Of the ten with extensive disease, six bad intracranial involvement. All patients were treated with surgery and only one received postoperative irradiation. Follow-up was available in 24, eight of which developed local recurrences (33%). All tumors, including recurrences and those with intracranial extension, were found to be diploid. We therefore conclude that tumor ploidy cannot be used to predict the clinical course of angiofibromas.

Journal Article↗

Complicated cholesteatomas: CT findings in inner ear complications of middle ear cholesteatomas.

Patients with facial palsy and middle ear disease, which may be chronic but clinically occult, may have a cholesteatoma with extension medially along the facial canal. In two patients, axial computed tomographic (CT) scans demonstrated involvement of the medial petrous bone. Patients with vertigo and chronic middle ear disease may have a cholesteatoma with a "fistula" between the middle and inner ears. Although the fistula usually involves the lateral semicircular canal, the cholesteatoma may pass through the oval window. In two patients, coronal CT scans showed extension to the oval window in one and through it in the other.

Adult↗

Surgical management of malignant tumors of the lacrimal gland.

Malignant lacrimal gland tumors are rare but serious and life-threatening neoplasms. Patients frequently present with symptoms of less than 1 year duration, often demonstrating rapid change during the 6 months preceding diagnosis. Pain and periorbital sensory changes are also reported by patients. Radiographically, bone destruction is a strong indication of a lacrimal gland malignancy as is ophthalmoplegia. Radical three-dimensional resection utilizing a cranioorbital approach offers the best chance for total tumor removal. Immediate reconstruction with a scalp flap is essential for primary healing, as well as esthetic balance of the orbital region.

Adenocarcinoma↗

Autogenous vein graft as a conduit for nerve regeneration.

This study was performed to determine whether vein grafts might serve as a conduit for nerve regeneration. A 1 cm segment of sciatic nerve was removed bilaterally in 12 Sprague-Dawley rats. On one side the gap was not repaired, and on the other side a segment of femoral vein was used to bridge the nerve gap. Nerve conduction studies and necropsies were performed at intervals. Reconstitution of nerve trunk continuity and healing of plantar ulcers occurred only in the vein-grafted side. Histologic examination revealed orderly growth of nerve fibers within the lumen of the vein grafts as early as 1 month after repair. Most regenerating nerve fibers passed through the proximal junction in an orderly pattern and reached the distal stumps within 2 months after repair. Results of nerve conduction study at 4 months after operation demonstrated restoration of conduction through the vein-grafted sciatic nerves with muscle reinnervation. Nearly normal muscle fibers in the gastrocnemius on the repaired side were confirmed at necropsy. This study demonstrated that autogenous vein grafts can serve as a conduit for nerve regeneration in rodents.

Animals↗

Role of microsurgery in orchiopexy.

Intra-abdominal tests cannot be completely brought down into the scrotum by conventional methods. Four intra-abdominal testes in 3 patients underwent successful orchiopexy by dividing the spermatic artery and vein near their origin and anastomosing these vessels to the inferior epigastric vessels with the use of microsurgical technique.

Child↗