Simultaneous leukaemic infiltrate and cholesteatoma in the same temporal bone

An otherwise healthy 37-year-old man came to the emergency room with left-sided dull chest pain of 4 weeks' duration. Physical examination, laboratory studies, and electrocardiogram were all unremarkable. A chest x-ray film revealed calcified pulmonary nodules. Computed tomography of the chest confirmed bilateral parenchymal cavitary lesions. Via limited thoracotomy, a tan nodule measuring 2.5 to 3.0 cm in diameter was excised from the left upper lobe. Histopathologic examination revealed a well circumscribed lesion and extensive lamellar hyalinization. A few foci of finely granular calcification were present within the hyalinizing areas. After surgery and short-term use of nonnarcotic analgesics, the chest pain resolved. Although pulmonary hyalinizing granuloma (PHG) is known to produce cavitating lesions, calcification at multiple sites is also consistent with this diagnosis. Clinicians should remember to include PHG in the differential diagnosis of multiple pulmonary nodules.     

Meningiomas of the temporal bone

Meningiomas should be considered in the differential diagnosis of otologic tumors arising from the middle ear and mastoid. It is generally recognized that meningiomas may invade the temporal bone via direct extension from the intracranial space (secondary). It is not generally recognized that meningiomas can develop initially in the middle ear cleft (primary). Representative cases of primary and secondary meningiomas of the temporal bone are described and clinical aspects of diagnosis surgical management are discussed. In order to assess the pathogenesis of meningiomas of the temporal bone, histological studies were done on 200 temporal bone specimens. In certain specimens, ectopic arachnoid granulations, from which primary meningiomas may originate, were identified and described, according to location. One specimen was most unusual in that it contained ectopic glial as well as arachnoidal tissue.     

Anatomy of the temporal bone

High resolution computed tomography has proved to be invaluable in the evaluation of the temporal bone, and demonstrates its bony anatomy in exquisite detail. Furthermore, the role of magnetic resonance imaging, especially with improving high resolution techniques, has continued to expand in the past decade. A thorough understanding of normal temporal bone anatomy is a requisite for accurate radiologic evaluation. The ensuing discussion is an attempt to summarize the normal temporal bone anatomy with emphasis on clinically relevant structures, temporal bone pneumatization, and common anatomic variants.     

Congenital cholesteatoma in a child carrying a gene mutation for adenomatous polyposis coli

A 53-year-old woman with a history of cervical carcinoma 14 years ago, treated with hysterectomy and radiation therapy, was admitted to the intensive care unit with severe SIRS (systemic inflammatory response syndrome) progressing to shock, multiple organ failure and death within 5 d. Bilateral hydronephrosis diagnosed by sonography and an enlarged left kidney with suspected abscesses verified in a CT-scan suggested the diagnosis of urosepsis. However, multiple microbiological examinations remained sterile. Despite surgical treatment and aggressive intensive care, she died in unresponsive shock. Pathohistologically, an angiotropic large B-cell lymphoma, a rare diffuse intravascular neoplasm of lymphoid origin, was diagnosed. The patient's history of abdominal radiation therapy 14 years earlier as well as multiple negative microbiological specimens in a patient with suspected urosepsis should have initiated the search for a non-infectious cause of the disease.     

Glomus tumor of the temporal bone

A case story describing the typical symptoms and course of a glomus tumour of the temporal bone is presented. The most frequent symptoms are pulsatile tinnitus, unilateral hearing loss, aural fullness and paresis of the vagal nerve or other lower cranial nerves. The tumour is frequently visible by otoscopy. Despite being histologically benign, the tumour is infiltrative and may affect the surrounding cranial nerves or spread into the cranial cavity. The early signs and findings are vague. Since the sequelae are fewer when the tumour is treated while it is small, an increased awareness will be of benefit to the patients.     

Osteoclastoma of temporal and zygomatic bone

A twelve year boy was admitted to the Otolaryngological Clinic of the Medical Academy in Cracow with a paralysis of the peripheral facial nerve and a tumor on the right zygomatic arc. A CT investigation revealed an expansive process in the central fossa of the skull, which continuously passed into the squama of the temporal bone and the are of the zygomatic bone on the right side, causing a paralysis of the nerve VII. A neurosurgical intervention was carried out as well as a subsequent treatment by radiotherapy. A remission of the tumor was achieved. Osteoclastoma was diagnosed histologically.     

Desmoplastic fibroma of the temporal bone

BACKGROUND: Desmoplastic fibroma is a benign, locally aggressive, intraosseous neoplasm with a propensity for local recurrence. Desmoplastic fibroma most commonly originates within the mandible (70% of cases), and long bones with rare lesions reported in the maxillary, frontal, and parietal bones. We report two patients with desmoplastic fibroma arising within the temporal bone. STUDY DESIGN: Case report and literature review. PATIENTS: Two patients with desmoplastic fibroma with intracranial and extra cranial growth patterns are presented. Both were young females with aural fullness and decreased hearing. INTERVENTIONS: Diagnostic and therapeutic. RESULTS: Both tumors were surgically extirpated and the patients have remained disease free for 18-48 months. Neither tumor expressed estrogen or progesterone receptors. CONCLUSION: Desmoplastic fibroma is a highly invasive local destructive lesion which is best treated by nondestructive surgical intervention. Immunohistochemical analysis may demonstrate hormonal receptors, in which case Tamoxifen may reduce recurrence.     

Congenital ectopic nail with bone deformity

We have presented a case of fulminating TEN with a fatal outcome. We believe there is strong probability that the TEN was caused by a propionic acid NSAID oxaprozin. This is the first reported case of TEN related to this particular agent. Toxic epidermal necrolysis has been reported with all types of NSAIDs. It appears from this case that switching from one class of nonsteroidal anti-inflammatories to another is not always without risk. Despite the class of nonsteroidal anti-inflammatory agent used, the possibility of systemic reaction cannot be excluded.     

Giant cell tumor and temporal bone

Receptors for regulatory peptides such as somatostatin or vasoactive intestinal polypeptide are expressed by a number of human neoplasms and can be visualized in vivo with peptide receptor scintigraphy. Recently, the CCK-B receptor, which binds both gastrin and cholecystokinin with high affinity, was shown using in vitro methods to be overexpressed in a number of human tumor tissues, including medullary thyroid carcinomas, small cell lung cancers, astrocytomas, gastrointestinal tumors, and stromal ovarian cancers. In the present study, we have designed novel, unsulfated CCK octapeptide analogs linked to the metal chelating DTPA and DOTA, and have tested them for their binding affinity to CCK-B receptor-positive tissue from human tumors: The most potent compounds assayed were DTPA-[Nle28, 31]-CCK(26-33) (MP2286) and DTPA-[d-Asp26,Nle28,31]-CCK(26-33) (MP2288) with an IC50 of 1.5 nM. For comparison, analogs with C-terminal DTPA, such as [Nle28,31,Aphe33(p-NH-DTPA)]-CCK(26-33) and CCK-(26-33)-NH(CH2)2 NH-DTPA, had an IC50 of >100 nM. DOTA-[D-Asp26, Nle28,31]-CCK(26-33) had an IC50 of 3.9 nM. The compounds were selective for CCK-B receptors as they did not bind with high affinity to CCK-A receptors expressed in human tumors (meningiomas or gastroenteropancreatic tumors). In vivo rat biodistribution studies with indium-111 labeled MP2286 and MP2288 showed that the primary mode of clearance was renal, and the primary sites of uptake (% ID/g 24 h p.i.) were kidneys (0.270 and 0.262, respectively) and the gastrointestinal tract. The CCK-B receptor-expressing gastric mucosa showed specific in vivo accumulation of 111In-labeled MP2288 which could be blocked in the presence of excess unlabeled MP2288. 111In-labeled MP2286 and MP2288 were also found to be stable in human plasma whereas both compounds were degraded in urine (>40% after 3 h at 37 degrees C). The affinity, specificity, biodistribution, and stability of these two DTPA-CCK analogs indicate that these compounds have substantial promise for use in the in vivo visualization of CCK-B receptor-expressing tumors.     

Acute intracranial complications of temporal bone trauma

BACKGROUND: A total of 110 patients, in whom kidneys from 95 living related and 15 cadaver donor, had experienced renal transplantation between February 1985 and October 1996 in our clinic. This study was conducted to evaluate the influence of the various pre-operative factors to the graft survivals and clinical course of patients in living related renal transplantation. METHODS: In 95 recipients, 17 adult patients had long term graft survivals over 5 years including 6 recurrent or denovo nephritis without chronic allografts nephropathy. Eight failed to graft loss attributed to chronic allografts nephropathy diagnosed within 5 years. Retrospective analysis were performed to elucidate the differences of these recipients. RESULTS: Donors of long graft survival recipients were younger (49.1 +/- 12.1 v.s. 58.9 +/- 10. 2) and had a better renal function evaluated by preoperative creatinine clearance in living related donors (115.5 +/- 37.0 v.s. 79.7 +/- 22.0 1/day). Graft long survival recipients had experienced less frequencies of acute rejection within 6 months (0.53 +/- 0.62: 8 patients, 9 times) compared with chronic allografts nephropathy recipients (1.00 +/- 0.53: 7 patients, 8 times). Long graft survival recipients had better responses to the antirejection therapy. Additionally acute rejection over 6 months were experienced only in chronic allografts nephropathy recipients. Higher serum creatinine level was revealed in recipients with chronic allografts nephropathy at 1 year after transplantation (1.27 +/- 0.27 v.s. 1.88 +/- 0.42 mg/dl). CONCLUSIONS: We concluded that donor age and renal function are related to the graft long survival as background factors. Long graft survival recipients had less frequency of acute rejection and good response to the antirejection therapy. In recipients with of acute rejection and good response to the antirejection therapy. In recipients with chronic allografts nephropathy, serum cretine level had already increased gradually within 1 year.     

Inflammations and tumors of the temporal bone

The term 'inflammation of the middle ear' covers a couple of diseases which range from the acute otitis media to the middle ear cholesteatoma. However, a clear characterization of a certain pathology is essential for any further treatment. Therefore this article presents a short overview about the different types of infections and their clinical manifestation. The tumors of the temporal bone show a great variety in their incidence. Even if tumors like the acoustic neurinoma or the paraganglioma are comparatively common, the chondroblastoma of the temporal bone is absolutely rare. In spite of these differences the individual temporal bone neoplasias are shortly mentioned herein.     

Infantile myofibromatosis located in the temporal bone

Infantile myofibromatosis (IM) is a proliferative disorder of infancy and early childhood characterized by the nodular or diffuse growth of lesions that are comprised of a mixture of mesenchymal elements. Intracranial involvement is reportedly rare, only eight such patients having been reported to our knowledge. We report on a 4-year-old boy with intracranial IM with a mass in his left temporal bone. A previous report on intracranial IM proposed that the underlying dura mater should be resected because of the possibility of early recurrence. At surgery in this case, the tumor was noted to be located in the bone itself and did not arise from the underlying dura. Therefore, the underlying dura mater and venous sinus were preserved. The follow-up MRI showed no sign of recurrences. It may not to be necessary to resect the dura mater in patients with intracranial IM.     

3-D morphological study of the temporal bone

OBJECTIVE: To establish the 3-D(three-dimensional) morphological study of the temporal bone by means of computer graphic techniques. METHODS: The serial sections of the temporal bone were processed by the technique of computer-aided 3-D reconstruction. The 3-D images of the multi-structures in the temporal bone were displayed on the monitor. The 3-D parameters of these structures were measured by a special software. The stereo-images of the structures in the temporal bone were obtained by stereoscopy and stereo-pairs. RESULTS: Most structures of the temporal bone were reconstructed in 37 instances for the different purpose of study. Each set of the stereo-pair corresponding to the structures in the temporal bones and many 3-D parameters were obtained. The complex spatial relationship among the reconstructed structures such as the facial nerve, endolymphatic sac, posterior tympanum and posterior ampullary nerve was revealed and the mechanic model of the ossicular chain was set up. According to these results, the surgical approach of the posterior ampullary nerve transection was designed and simulated on the graphic computer. CONCLUSION: The technique of computer-aided 3-D reconstruction provides a new tool for the study of the temporal bone. It is also helpful for the designs and simulations of the surgical approaches. The results of this study contribute to developmout of a new branch of pathology of the temporal bone and a primary 3-D morphological study of the temporal bone.     

Parapharyngeal emphysema--an unusual complication of temporal bone trauma

Medical cure of fungal prosthetic valve endocarditis (PVE) is rarely reported. We describe a patient with C. tropicalis PVE in whom surgery was believed to be contraindicated. A huge tricuspid valvular vegetation was identified by two-dimensional transthoracic echocardiography. After a total of 2 g of amphotericin B, she continued with fluconazole daily with a follow-up of fifteen months. Our patient represents the first case of long term survival of C. tropicalis PVE successfully managed without surgery.     

Congenital erythropoietic porphyria successfully treated by allogeneic bone marrow transplantation

The long-term biochemical and clinical effectiveness of allogenic bone marrow transplantation (BMT) was shown in a severely affected, transfusion-dependent 18-month-old female with congenital erythropoietic porphyria (CEP), an autosomal recessive inborn error of heme biosynthesis resulting from mutations in the uroporphyrinogen III synthase (URO-synthase) gene. Three years post-BMT, the recipient had normal hemoglobin, markedly reduced urinary porphyrin excretion, and no cutaneous lesions with unlimited exposure to sunlight. The patient was homoallelic for a novel URO-synthase missense mutation, G188R, that expressed less than 5% of mean normal activity in Escherichia coli, consistent with her transfusion dependency. Because the clinical severity of CEP is highly variable, ranging from nonimmune hydrops fetalis to milder, later onset forms with only cutaneous lesions, the importance of genotyping newly diagnosed infants to select severely affected patients for BMT is emphasized. In addition, the long-term effectiveness of BMT in this patient provides the rationale for future hematopoietic stem cell gene therapy in severely affected patients with CEP.