Pleomorphic CD8+ small/medium size cutaneous T-cell lymphoma.

Pleomorphic small/medium-sized cutaneous T-cell lymphoma is a recently recognized rare type of cutaneous T-cell lymphoma which is clinicopathologically different from mycosis fungoides and Sezary syndrome. By definition the phenotype of the neoplastic lymphocytes in pleomorphic small/medium-sized cutaneous CD3CD4CD8 but CD8 pleomorphic small/medium sized cutaneous T-cell lymphoma cases have been occasionally described. We describe a 55-year-old female with a pruritic erythematous nodule on the lateral aspect of her right foot present for 1.5 years. Histology revealed a nonepidermotropic lichenoid infiltrate in the papillary dermis and a patchy infiltrate in the mid and lower dermis composed of small to medium-sized pleomorphic lymphocytes. The immunophenotype of these lymphocytes was CD3CD4CD8TIA-1. Staining for CD20, CD30, CD56, TdT, and LMP1 were negative, and the Ki-67 proliferation index was 5% to 10%. Gene rearrangement studies demonstrated a T-cell clone. The laboratory and imaging workup did not reveal extracutaneous involvement. The lesion was treated by local irradiation but a follow-up biopsy demonstrated only partial remission. Consequently, the lesion was treated by surgical excision.

Insect bite-like reaction associated with mantle cell lymphoma: clinicopathological, immunopathological, and molecular studies.

A cutaneous eruption simulating insect bites has been repeatedly described in association with chronic lymphocytic leukemia (CLL). It was only rarely described with mantle cell lymphoma (MCL). Our study was performed to elucidate the clinical, histologic, immunopathological, and molecular characteristics of insect bite like reaction (IBLR) associated with MCL. The clinical presentation and histologic findings in 3 IBLR cases associated with MCL were found to be similar to 3 IBLR cases associated with CLL. The eruptions consisted of itchy erythematous papules, nodules, plaques, and vesicles. Non-vesicular lesions were characterized histologically by normal or mildly spongiotic epidermis. Vesicular lesions were characterized by marked spongiosis and intraepidermal spongiotic vesicles containing eosinophils, or marked subepidermal edema occasionally leading to a dermoepidermal separation. Most of the lesions were characterized by superficial and mid dermal to deep perivascular and interstitial, and occasionally periadnexal, inflammatory-cell infiltrate consisting of mononuclear cells and eosinophils. The densities of the infiltrates varied and the inflammatory-cell infiltrate extended often into the fat lobules. Neutrophils and nuclear dust were found more frequently and abundantly in the IBLR lesions associated with MCL. Immunophenotyping, direct immunofluorescence (DIF) tests, and IgH gene rearrangement studies were performed in the lesions associated with MCL only. The majority of the infiltrating lymphocytes were CD3+, CD5+ and CD43+, more CD4+ than CD8+, and only a small minority was CD20+. The cells did not stain for bcl-1 protein and CD30, and with no evidence of clonality. The DIF test result was negative. The IBLR eruption associated with MCL resembles clinically and histologically IBLR associated with CLL. The eruption seems to be reactive rather than neoplastic, because there is no evidence of MCL involvement in the skin lesions.

Case report of regional alveolar bone actinomycosis: a juvenile periodontitis-like lesion.

BACKGROUND: Cervicofacial actinomycosis infection most often involves the mandibular bone and rarely the alveolar crest. METHODS: We describe a 14-year-old patient who had actinomycosis involving the alveolar bone at the left lower dental quadrant region. Resembling juvenile periodontitis, it was difficult to diagnose properly and resulted in devastating dental and periodontal consequences: loss of one tooth with most of its adjacent regional alveolar bone, severely compromising the support of two other teeth. RESULTS: With the diagnosis came successful treatment, including surgical removal of the soft and hard tissues with concomitant prolonged penicillin administration. CONCLUSIONS: We feel that this case should raise the interest and concern of both the periodontist and the general practitioner so that early diagnosis can be obtained, significantly improving the clinical outcome.

Sarcoma of the maxillofacial region: follow-up of 25 cases.

BACKGROUND: Maxillofacial sarcomas are rare tumors, varying according to their type, grade, site of occurrence, response to treatment and prognosis. Approximately 10% of all cases of osteosarcoma, a subtype with a particularly poor prognosis, occur in the head and neck. PATIENTS AND METHODS: During the 35 years from 1963 until 1997, the 25 patients treated for sarcomas at our center were identified and evaluated according to a variety of parameters. RESULTS: Overall 2- and 5-year survival rates were 72% and 60%, respectively. Sarcomas were found most commonly in the mandible, maxilla and tongue. Survival was not significantly correlated with gender, ethnic origin or histopathological staging, but significant correlation was found between type of sarcoma and survival whilst younger patients had better survival rates. CONCLUSION: Advanced facial reconstruction methods may enable the performance of more radical ablative surgery, thereby improving survival outcome for head and neck osteosarcoma patients. Osteosarcoma appears to be as lethal in the maxillofacial region as in the extremities.

Generalized periodontal involvement in a young patient with systemic lupus erythematosus.

Inflammation is considered to be a leading cause of morbidity in systemic lupus erythematosus (SLE), yet inflammatory periodontal involvement is rarely encountered. A young lady suffering from active SLE accompanied by severe periodontal loss, manifested by gingival recession of all her teeth, was referred to our clinic for treatment. The association between periodontal involvement and connective tissue diseases is unclear, and the literature dealing with periodontal involvement in patients suffering from Sjogren's syndrome and rheumatoid arthritis is comprised of studies showing both normal and pathological periodontal status. We discuss the possible underlying mechanisms.

Celiac disease-associated alopecia in childhood.

We report the association of celiac disease and alopecia in 3 children. In one, the alopecia developed after 4 years' nonadherence to a gluten-free diet; the other 2 patients presented with alopecia. Administration of a gluten-free diet resulted in partial regrowth of hair in the first child and complete hair growth in the others.

T cell lymphoma of the ear presenting as mastoiditis.

Mastoiditis is a complication of otitis media characterized by suppuration and destruction of air cell septa in the mastoid and petrous pyramid. Diagnosis is made by clinical findings and computerized tomography (CT) of the temporal bone. We present a patient initially diagnosed by CT as having chronic mastoiditis who was subsequently shown to have an unusual large-cell malignant lymphoma of T cell type.

Severe humoral hypercalcemia in primary isolated non-Hodgkin's lymphoma of the heart.

The etiology of hypercalcemia was investigated in a patient with primary isolated non-Hodgkin's lymphoma of the heart. There was no evidence of bone involvement, and parathyroid hormone and calciterol levels were suppressed. Plasma parathyroid-hormone-related protein (PTHrP 1-86) detected by immunoradiometric assay was increased (15 pmol/l compared with < 0.3 pmol/l in a control). We demonstrated that PTHrP was the humoral mediator of severe hypercalcemia in our patient.

Chemotherapy-induced spontaneous pneumothorax in a patient with bulky mediastinal lymphoma: a rare oncologic emergency.

Chemotherapy-induced spontaneous pneumothorax (SP) has been described sporadically in chemosensitive tumors, particularly sarcoma, with multiple lung metastases. We present a patient who developed SP following rapid regression of bulky mediastinal lymphoma. Immediately on chest tube insertion, the lung recovered and further chemotherapy could be delivered uneventfully. We suggest that (1) chemotherapy-induced SP should be included amongst oncologic emergencies and that (2) a high degree of awareness of this complication is required.

Highly aggressive behaviour of occult papillary thyroid carcinoma.

Occult papillary thyroid carcinoma is generally associated with an excellent prognosis. Distant metastasis of this tumour is extremely rare. A case of occult papillary thyroid carcinoma with metastases to the lungs, cervical lymph nodes, skeleton, and the brain is reported. The tumour expressed itself in extremely aggressive clinical behaviour and responded only partially to aggressive therapy. The controversial methods of treatment for occult papillary thyroid carcinoma are also discussed.

Radiation-induced primary brain lymphoma: a case report.

A patient who developed primary brain lymphoma 6 years following whole brain irradiation due to a low-grade glioma is described. The patient had no evidence of congenital or acquired immunodeficiency state and achieved a good and prompt response to aggressive chemotherapy, including high-dose methotrexate. The previous radiation therapy is implicated in the etiology of the lymphoma because of the geometric coincidence, the relatively long latency period and the different histology. A brief review of current literature is reported.

Intermediate and high-grade gastric non-Hodgkin's lymphoma: a prospective study of non-surgical treatment with primary chemotherapy, with or without radiotherapy.

The role of surgery as initial treatment in gastric lymphoma remains controversial. We have prospectively evaluated a stomach conservation strategy in histologically aggressive gastric lymphoma, using primary adriamycin-containing chemotherapy, followed by involved-field radiotherapy in patients with limited disease. Twenty-six patients (median age 69 years) were entered in this study; 15 had stage I disease, 7 had stage II disease and 4 had stage IV disease. The chemotherapy combinations were CHOP (18 patients) and ProMACE/MOPP (8 patients). Radiotherapy was given to 11 patients. Of the 24 patients evaluated for response, 18 (75%) achieved endoscopically-confirmed complete response and 4 (17%) partial response. During follow-up (median 22 months), none of the complete responders developed recurrent lymphoma. Gastric resection was performed in 1/26 patients who did not respond to primary chemotherapy. There were no cases of perforation, but three patients (12%) developed acute gastro-intestinal bleeding a few days after the onset of chemotherapy, one of whom required a surgical devascularization procedure. There was no treatment-related mortality. These data further support the non-surgical approach in histologically aggressive gastric lymphoma, using primary chemotherapy with or without radiation therapy.

Primary Hodgkin's disease of the bone.

A primary extranodal presentation occurs in less than 0.25% of patients with Hodgkin's disease (HD) and we describe the primary clinicopathologic findings in a rare case of a 21-year-old female with primary bony involvement of Hodgkin's disease. The patient presented with microcytic, hypochromic anemia and shoulder pain. The diagnosis of HD (lymphocyte depleted type) was made by biopsy taken from a lytic lesion in the lateral third of the clavicle. Staging work-up revealed an additional lesion in the ilium, but there was no evidence of lymph node involvement. MOPP/ABV (nitrogen mustard, Oncovin, procarbazine, prednisone/adriamycin, bleomycin, and vinblastine) chemotherapy resulted in complete response lasting, to date, 36 months. To the best of our knowledge, this is only the second reported case of disseminated primary Hodgkin's disease of the bone and all have had a favourable response to combination chemotherapy. A review of the literature is also presented.

The effect of minocin on the pericardium.

The purpose of this study was to evaluate in a dog model whether intrapericardial instillation of tetracycline is superior to the effect of drainage alone in causing pericardial adhesions and cavity obliteration. Twelve mongrel dogs were randomly divided into two experimental groups. All dogs received a pericardial drain through a sterile thoracotomy. Group A dogs (n = 6) received minocycline hydrochloride, 20 mg/kg, group B received normal saline. All dogs were sacrificed 1 month later. Echocardiograms performed 1 week postsurgery were normal in all dogs. Macroscopic evaluation disclosed that all group A dogs had over 25% cavity obliteration whereas group B dogs had no adhesions or had obliteration of less than 25% of the cavity area. Microscopic evaluation showed that group A dogs had severe pericardial fibrosis and thickening with slight focal lymphoplasmocytic infiltration. Myocardial damage was not seen. Our results support the clinical use of injecting tetracycline into the pericardium (in addition to drainage) to cause cavity obliteration in patients with persistent pericardial effusion.

Necrotizing squamous metaplasia in herpetic tracheitis following prolonged intubation: a lesion similar to necrotizing sialometaplasia.

In the last year we have encountered three cases of clinically inapparent herpes simplex virus (HSV) tracheitis in biopsies from patients undergoing tracheostomy following prolonged intubation. The diagnosis was based on finding characteristic inclusion-bodies on haematoxylin and eosin stained sections and was confirmed by immunohistochemical staining of infected cells with antibodies to HSV. A prominent mononuclear infiltrate, originally described in HSV oesophagitis, was recognized in two cases. Diffuse squamous metaplasia of both surface and glandular epithelium adjacent to necrotic ulcerated areas was evident in all three cases. Where extensive, the necrotizing squamous metaplasia of the glandular/ductal component was similar to necrotizing sialometaplasia of salivary glands, and likewise simulated squamous or mucoepidermoid carcinoma.

Seatbelt injury to the common iliac artery: report of two cases and review of the literature.

Blunt trauma to the common iliac artery is rather rare. Moreover, seatbelt injuries to the common iliac artery have not yet been reported. This article presents two cases of seatbelt injury involving the common iliac artery. A deceleration-type mechanism is suggested as the primary cause of injury, resulting in the production of an intimal flap. The diagnosis of such an injury is based on clinical suspicion, a change of pulse, or lower limb ischemia. On arteriotomy the damage is greater than seen on external examination. Once diagnosed prompt treatment should follow to prevent loss of life or limb. The signs, symptoms, and modalities of treatment are reported, as well as a review of the literature.

Synovial sarcoma associated with osteofibrous dysplasia. A case report and review of the literature.

We report on a 14-year-old boy who demonstrated an unusual association between osteofibrous dysplasia and synovial sarcoma. This case suggests that a patient who presents with osteofibrous dysplasia of the tibia can eventually develop a malignant musculoskeletal tumor in the same anatomical location (same limb). This experience suggests that a patient who presents with osteofibrous dysplasia should be followed up for the possibility of a coexisting synovial sarcoma in the same leg.

Primary brain lymphoma after X-ray irradiation to the scalp for tinea capitis in childhood.

A 39-year-old male developed primary brain lymphoma 33 years after receiving scalp irradiation for tinea capitis. This is the first reported association between cranial irradiation during childhood and subsequent development of primary brain lymphoma.