Inflammatory myofibroblastic tumor of maxillary sinus successfully treated with radiotherapy and corticosteroid: report of a rare case.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a mesenchymal tumor mainly found in lung or retroperitoneum and rarely affects head and neck region. Extensive English literature search reveals that less than fifty cases of head and neck IMT have been reported so far, maxillary sinus being fewer. CASE PRESENTATION: We present a case of IMT involving maxillary sinus in a 48-year-old gentleman who attained complete clinico-radiologic response after treatment with radiotherapy (RT) and concurrent oral prednisolone. CONCLUSIONS: This is the first report where such magnificent response was attained in primary setting treated with RT and steroids as opposed to surgery which used to be considered as standard of care till now.

Inflammatory pseudotumor of the nasopharynx with spread along the trigeminal nerve.

Inflammatory pseudotumor of the nasopharynx is a rare diagnosis that is often misinterpreted as carcinoma or lymphoma. It has been referred to as a tumefactive fibroinflammatory lesion, idiopathic pseudotumor, and fibrosing inflammatory pseudotumor. We present a rare case of a 40-year-old African American female from Kenya with inflammatory pseudotumor of the nasopharynx with perineural spread of disease along the trigeminal nerve to discuss the diagnosis and treatment of such an uncommon entity.

Inflammatory pseudotumor presenting as a facial swelling.

We present a case of inflammatory pseudotumor (IPT) presenting as a facial swelling after an accidental hit on a right side of a face. As swelling did not resolve, dental examination and teeth extraction were done by a dentist presuming the swelling was misdiagnosed with infection of dental origin. Swelling grew even bigger and patient was referred to Department of Maxillofacial Surgery. CT scan of the face and FNA of the lesion was ordered. A homogenous tumor mass in the right infraorbital region in front of anterior wall of the maxillary sinus was seen on CT The result of the FNA was reactive hyperplasia of the lymph node. Since the lesion was easily accessible surgical exploration and complete extirpation was done. Pathohistological analysis indicated a low grade B-cell Non Hodgkin lymphoma. PCR showed policlonality of B cells discarding the diagnosis of lymphoma. Pathohistological review showed diffuse intramuscular, perineural and perivascular infiltration with small lymphocytes without formation of germinative centers. Imunohistochemistry was positive for CD20 and CD3. Taking into account all features the diagnosis of IPT was established. Diagnosis of IPT is a diagnosis by exclusion, combining clinical, radiological and pathohistological characteristics. Lack of clear histologic criteria makes differential diagnosis extremely difficult. Our case is unique regarding localisation of head & neck IPT no case presenting on the face in infraorbital region has been described in the literature. Although IPT is very rare in general and especially on the face, one should be aware of it when considering differential diagnosis of facial swelling.

Early-onset postirradiation sarcoma of the tongue after pseudotumor phase.

Radiation-induced sarcoma usually develops after an interval of more than 10 years from the completion of radiation therapy to the diagnosis of secondary sarcoma. However, the theory of radiation-induced transformation does not rule out postirradiation sarcomas with a short latency period. We experienced the case of a patient with postirradiation leiomyosarcoma of the tongue, which occurred 19 months after he had received chemoradiotherapy. Besides the short latency period, a pseudotumor stage developed between the time of radiation exposure and the development of leiomyosarcoma. In this article, we also describe an immunohistochemical approach to diagnose leiomyosarcoma and the efficacy of a gemcitabine and docetaxel regimen.

Inflammatory pseudotumor of mediastinum treated with tomotherapy and monitored with FDG-PET/CT: case report and literature review.

Mediastinal inflammatory pseudotumor is a rare disease with reactive pseudoneo-plastic features and a proven capacity for local invasion. The radiographic appearance of inflammatory pseudotumor is quite non-specific and the definitive diagnosis is based on the histological evaluation of tissue specimens. Resection of the lesion is the treatment of choice. However, nonsurgical treatments such as radiotherapy and steroids have been employed in the setting of incomplete surgical resection, tumor recurrence, and patients being unfit for surgery. The case described here is being reported because of the rare mediastinal location and atypical treatment approach including salvage irradiation and monitoring with FDG-PET/CT. Because of the irregular target volume inside the mediastinum as defined by FDG-PET/CT and the significant pulmonary comorbidity, it was deemed necessary to optimize dose delivery with intensity-modulated radiation therapy (IMRT). A possible gain by means of daily control of patient setup with image-guided radiation therapy was also hypothesized and we used tomotherapy to irradiate the lesion. The first FDG-PET/CT after treatment confirmed further reduction of the metabolic activity followed by stable disease in the mediastinum, with no new occurrence of disease 16, 24 and 30 months after tomotherapy.

Posterior fossa intracranial inflammatory pseudotumor: a case report and literature review.

BACKGROUND: Intracranial inflammatory pseudotumors are rare. This study describes an intracranial inflammatory pseudotumor at the left cerebellopontine angle. It is the second documented posterior fossa intracranial inflammatory pseudotumor, and it was treated by surgery and radiotherapy. CASE DESCRIPTION: A 49-year-old man presented with dizziness for 3 months and mild hoarseness for 1 month. Brain CT detected an intracranial tumor at the left cerebellopontine angle. Magnetic resonance imaging revealed a 3.6-cm heterogeneously enhancing mass. Suboccipital craniectomy with ventriculostomy was performed. The mass was well defined with a smooth surface, enclosed the low cranial nerves, and adhered to the dura matter. Pathologic examination revealed fibrous collagenous stroma with dense infiltrates of small lymphocytes and uninucleated histiocytes. Immunopositivity for T-200 and CD-68 was noted. Special staining for mycobacteria and fungus was negative. Serologic tests were positive for EBEA-Ab, EBNA-Ab, and EB-VCA-IgG. An inflammatory pseudotumor was diagnosed. Local recurrence was found 6 months later with a left oculomotor nerve palsy. Whole-brain irradiation with a total dose of 1200 cGy in 6 fractionations was done. Remission was found in follow-up neuroimages, and no recurrence was noted in 2 years' follow-up. CONCLUSION: Based on serologic findings and a literature review, the pathogenetic mechanism of this rare intracranial tumor is believed to be chronic reactive EBV infection. We propose that radiotherapy may be the best treatment option in the case of local recurrent intracranial inflammatory pseudotumors.

Non-epithelial lesions of the larynx: review of the 10-year experience in a tertiary Spanish hospital.

CONCLUSION: In this report we review the diagnosis, therapy, and outcome of laryngeal non-epithelial tumors and comment on the literature on these rare lesions. OBJECTIVES: Non-epithelial tumors of the larynx are rather rare and most cases have been reported as isolated or short series of cases from different centers all over the world. The aim of the present study was to review the 10-year experience with non-epithelial lesions in a 400-bed tertiary hospital covering a population of almost 250 000 people in Madrid, Spain. PATIENTS AND METHODS: We reviewed 2631 laryngeal samples corresponding to this time period. RESULTS: In all, 737 corresponded to carcinomas (726 squamous cell carcinomas). We had two cases of chondrosarcoma, one case of liposarcoma, one case of synovial sarcoma, and one neural benign tumor, suggestive of neurinoma. In this period we also had an inflammatory pseudotumor affecting the vocal cord, a case of primary laryngeal extramedullary plasmocytoma, and one case of high grade diffuse malignant lymphoma with exclusive involvement of the larynx. In our series most patients were women (five vs three patients) and ages ranged between 12 and 92 years.

Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy.

OBJECTIVE: To evaluate the response to steroid and radiation therapy in inflammatory pseudotumor (IPT) involving the skull base. STUDY DESIGN AND SETTING: Eight patients with IPT of the skull base were evaluated. Six patients were diabetic. All patients underwent initial high-dose steroid therapy. Seven of 8 patients underwent subsequent low-dose steroid maintenance. Additional low-dose radiation therapy was performed in 6 patients. We reviewed the medical records and radiologic findings of the patients to evaluate their responses to the steroid and radiation therapy. RESULTS: In all patients, the initial response to steroid therapy was fair. However, 7 patients showed symptom recurrence after cessation of steroid therapy. In 5 of 6 patients, low-dose radiation therapy resulted in recurrence. On follow-up MRI, none of the patients showed complete remission of the disease. CONCLUSION: This study shows aggressive clinical behavior of IPT involving the skull base and its poor response to steroid therapy and low-dose radiation therapy. For improving control, more aggressive initial efforts including high-dose radiation therapy with or without concurrent steroid therapy might play a role. EBM RATING: C-4.

[Imaging features of lung carcinoma, pulmonary tuberculoma, and inflammatory pseudotumor on helical incremental dynamic CT scan--a report of 44 cases].

BACKGROUND & OBJECTIVE: Lung carcinoma, pulmonary tuberculoma, and inflammatory pesudotumor are the most common solitary pulmonary nodules (SPN). Computed tomography (CT) is the major diagnostic examination of them. Most recent reports focused on their morphologic features on CT. The differential diagnoses are still difficult. This study was to explore their imaging features on helical incremental dynamic CT scan according to the differences of their hematal supply, and get more information for the diagnosis and differential diagnosis. METHODS: Incremental dynamic CT scan was performed on 115 cases of solitary pulmonary nodule. Of the 44 cases proved by postoperative pathology, 30 were lung carcinoma, 7 were pulmonary tuberculoma, and 7 were inflammatory pesudotumor. RESULTS: Of the 30 cases of lung carcinoma, 7 were mildly enhanced, 14 were moderately enhanced, 5 were highly enhanced, 2 were remarkably enhanced, and 7 were not enhanced; 9 had peak enhancement began at 18-28 s after injection, 17 began at 30-39 s, and 4 began at 40-44 s, with an average time of 32 s. Of the 7 cases of tuberculoma, 5 were not enhanced, and 2 were cricoid enhanced. All the 7 cases of inflammatory pesudotumor were remarkably enhanced, with the peak enhancement value of over 100 Hu in all cases; the peak enhancements began at 26 s, 28 s, 30 s, 32 s, 33 s, 110 s, and 122 s, respectively, after injection. CONCLUSION: Helical incremental dynamic CT is helpful in differential diagnoses of lung carcinoma, pulmonary tuberculoma, and inflammatory pesudotumor.

Meningeal plasma cell granuloma with relapsing polychondritis. Case report.

Relapsing polychondritis (RP) is a rare systemic disease characterized by recurrent inflammation of the cartilaginous structures and connective tissue. Central nervous system lesions in association with RP have occasionally been reported, but intracranial mass lesions have not been described. The authors report the first such case, in which a 51-year-old man presented with parasagittal meningeal plasma cell granuloma with RP. The mass was subtotally resected and adjuvant radiotherapy was administered. The patient did not experience any recurrence of the lesion during an 8-year follow-up period. In this case, the exact diagnosis of RP was made based on symptoms of respiratory tract chondritis, which was successfully treated by the placement of tracheobronchial stents.

Plasma cell granuloma--an enigmatic lesion: description of an extensive intracranial case and review of the literature.

We present an unusual case of intracranial plasma cell granuloma in a 70-year-old man with a 6-month history of progressive visual disturbance. The lesion extensively involved the cranial base, extended into the frontal region, and reached the floor of the third ventricle in the suprasellar area. Microscopic examination of multiple diagnostic transsphenoidal biopsies showed an inflammatory proliferation with a predominance of cells that were immunohistochemically determined to be polyclonal plasma cells. Ultrastructural analysis confirmed the presence of numerous mature plasma cells in a mixed inflammatory proliferation. In situ hybridization for Epstein-Barr viral RNA revealed no evidence of viral expression. The patient was treated with steroid therapy and radiotherapy, without any appreciable reduction of the lesion's size. He is alive with persistent severe visual disturbance 14 months after the diagnosis. We discuss the etiopathogenetic, diagnostic, and therapeutic issues related to this entity, and review the literature.

Invasive inflammatory pseudo-tumor involving the lung and the mediastinum.

Inflammatory pseudotumor is a rare clinical entity. We report here the case of a 14-year-old boy who underwent lung resection en bloc with the lower esophagus, the mediastinal pleura and the diaphragm for an inflammatory pseudotumor of the left lower lobe. Postoperative radiotherapy was administered. The patient is well at 9-year follow-up. Inflammatory pseudotumor may present a particularly aggressive behavior. Multimodality approach, including extensive surgical resection, may be necessary in dealing with this disease.

Double malignant neoplasms occurring long after local radiation to the oral mucosa.

A 59-year-old woman who had received cobalt-60 (60Co) interstitial radiation therapy (total 44 Gy) in the right bucco-gingival region for inflammatory pseudotumour was found to have metachronous double malignant neoplasms. Initial osteosarcoma of the right mandibular angle and subsequent squamous cell carcinoma of the right buccal mucosa were identified 28 and 33 years after the radiation, respectively. Since both tumours were located very close to the focus of previous radiation, the therapy was considered to be responsible for their genesis. The patient had systemic metastases of the osteosarcoma.

The surgical management of lacrimal gland pseudotumors.

PURPOSE: Lacrimal gland pseudotumors belong to the group of orbital pseudotumor. Systemic corticosteroids are advocated as the primary treatment of choice in orbital pseudotumor, but recurrent and refractory cases are commonly described. In this retrospective study, the authors evaluate alteerative treatment options such as surgical excision or debulking of lacrimal gland pseudotumors. METHODS: The records of 26 patients referred to the Orbital Center of Amsterdam between 1976 and 1994 with a diagnosis of lacrimal gland pseudotumor were reviewed with special reference to computed tomography scans, histopathologic specimens (in 23 patients), treatment regimens, and final clinical outcome. RESULTS: Histopathologic review showed 15 nonsclerosing (classic) and 8 sclerosing lacrimal gland pseudotumors. Initial treatment consisted of corticosteroids alone (9/26), surgery alone (7/26), a combination of surgery and irradiation (5/26), a combination of surgery and corticosteroids (4/26), or indomethacin alone (1/26). Surgery comprised tumor excision or tumor debulking. Of the patients treated with corticosteroids alone, 55% (5/9) responded initially but only 22% (2/9) obtained a cure. However, all patients treated with surgery combined with corticosteroids/irradiation (9/9), with surgery alone (7/7), or with indomethacin alone (1/1) responded well without recurrences. The main complication of therapy was dry eye syndrome, the incidence being highest in patients who received surgery of the palpebral lobe or irradiation. The mean follow-up was 4.9 years. CONCLUSION: Surgical excision or debulking is a safe and effective treatment option in lacrimal gland pseudotumors, even in the histopathologic sclerosing variant.

Plasma cell granuloma of the temporal bone.

Plasma cell granulomas are uncommon, benign tumors comprised primarily of mature plasma cells and are a distinct entity, separate from plasmacytomas. They are rarely found in the head and neck, and have never been reported in the temporal bone. This case represents the first report of a plasma cell granuloma originating in the temporal bone. Treatment options include surgical removal and radiation therapy.

Plasma cell granuloma of the supraglottic larynx.

The occurrence of plasma cell granuloma of the larynx appears to be unusual. Review of the literature revealed only two previously reported cases. We present an additional case of plasma cell granuloma of the larynx. The diagnosis was made by histological and immunohistochemical examinations. The tumour was successfully treated by radiation therapy.

Intracranial plasma cell granuloma evaluated by positron emission tomography.

A 50-year-old farmer with plasma cell granuloma had PET scans showing marked accumulation of C-11 Met. The PET had superiority in imaging the existence and extent of the lesion and monitored therapeutic response as compared with CT and MR imaging. PET yields information on hemocirculatory and metabolic aspects of this rare disease.

Plasma cell granuloma of the nasal cavity treated by radiation therapy.

Plasma cell granuloma is a rare, benign tumor most commonly found in the lungs in patients younger than 30 years. Although presentation has been reported at a number of other anatomic sites, this report is the first of plasma cell granuloma of the nasal cavity. The tumor was initially resected, but progression was seen at 1-month follow-up. Because further surgery to completely eradicate the tumor would have been extensive and disfiguring, 40-Gy external beam radiation was given in 20 fractions using a three-field wedge technique. Most recent clinical follow-up at 27 months showed local control. Surgery remains the treatment of choice for plasma cell granuloma when the disease can be completely resected. However, irradiation can also be effective in patients with recurrent or inoperable local disease.

Plasma cell granuloma of the hypothalamic region.

Two patients suffering from diabetes insipidus, with additional symptoms of impaired vision and signs of panhypopituitarism and increased intracranial pressure, showed a normal sella tursica in the X-ray examination of the skull but large, dense space-occupying lesions in the hypothalamus on CT scans of the brain. NMR performed in one patient disclosed suprasellar growth of a hypothalamic lesion. Proliferation of lymphoplasmocytes and mature plasma cells was seen by light microscopic and electromicroscopical examination of biopsy samples in both cases; histiocytes and multinucleated giant cells were absent; tuberculosis, syphilis and sarcoidosis were ruled out by appropriate tests. Plasma cells exhibited polyclonal immunoglobulin expression as revealed by immunocytochemistry using the PAP method. Taken together these features are typical of plasma cell granuloma. Transitory remission after radiotherapy was obtained in one patient.

Primary intracranial plasma-cell granuloma. Case report.

The authors report the fourth case of primary intracranial plasma-cell granuloma. The patient was a 16-year-old girl who presented with loss of vision as the major clinical feature. The tumor resembled a meningioma both preoperatively and grossly at surgery. Because the tumor did not respond to steroid treatment following subtotal surgical excision, radiation therapy was administered to the affected area. Major considerations in the differential diagnosis of this neoplasm are discussed.