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1/11. Anaplastic large cell lymphoma associated with sjogren's syndrome.

    We report a case of a 20-year-old Japanese female with recurrent anaplastic large cell lymphoma (ALCL) associated with sjogren's syndrome (SjS). She was first diagnosed to have ALCL presenting with axillary lymphadenopathy, which within a month underwent spontaneous remission, at the age of 12 years. Eight years later she developed left inguinal lymphadenopathy with clinical overt sicca symptoms associated with elevated serum IgG, interleukin (IL)-1beta and IL-6 levels. Lymph node biopsy was now diagnostic of ALCL characterized by large pleomorphic CD30 blast cells with the specific chromosomal abnormality, t(2;5)(p23;q35). In contrast to this the salivary gland and renal biopsy revealed infiltration of small lymphocytes, morphologically and cytogenetically distinct from the ALCL cells. Interestingly, SjS symptomatology correlated with disease activity of ALCL and based on an association with elevated IgG and IL-6 levels, suggesting that the concurrence of these two diseases could be more than coincidental. To the best of our knowledge, this is the first reported case of ALCL presenting concurrently with SjS.
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2/11. Tufted angioma of adult onset, revealing abundant eccrine glands and central regression.

    Tufted angioma (angioblastoma) usually occurs in infancy. Some lesions reveal proliferation of eccrine glands and some undergo spontaneous regression. We report a lesion from the left lateral chest of a 58-year-old male, which had appeared 5 years ago and gradually increased in size. Its color had faded out centrally. Histological examination showed many angiomatous lobules on the border of the lesion but few in the central portion. Abundant eccrine sweat glands, small venules and dense connective tissue were seen in both areas. The histology and the clinical course suggested that central regression occurred in the lesion. Out of 211 reported cases, we found 4 cases that had annular lesions spreading centrifugally. Though these lesions were not examined histologically, they were suspected to regress in the central portions. We suggest that central regression can rarely occur in tufted angioma.
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3/11. Transient spontaneous regression of aggressive non-Hodgkin's lymphoma confined to the adrenal glands.

    A 72-year-old-man with night sweats and a low-grade fever was found to have bilateral adrenal enlargement associated with incipient adrenal insufficiency. Without any intervention, these adrenal lesions regressed spontaneously, accompanied by disappearance of clinical symptoms. Seven months later, however, the lesions became enlarged and exceeded their initial size while remaining confined to the adrenals, associated with reappearance of nights sweats and overt adrenal insufficiency. Upon unilateral laparoscopic adrenalectomy, the lesion proved histopathologically to be diffuse large B cell non-Hodgkin's lymphoma (NHL). After contralateral laparoscopic adrenalectomy and adjuvant chemotherapy, the patient remains alive with no recurrence at 26 months. Treatment with bilateral adrenalectomy and chemotherapy is effective for aggressive NHL confined to bilateral adrenal glands. A transient clinical improvement without treatment was considered to be due to a spontaneous regression of NHL, although we could not confirm the histological results before the regression. Such tumor behavior showing spontaneous regression in spite of aggressive histology may also be related to the favorable outcome, even though regression was transient.
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4/11. Spontaneous regression of metastatic malignant melanoma of the parotid gland and neck lymph nodes: a case report and a review of the literature.

    We report the case history of a patient with complete spontaneous regression of metastatic cutaneous melanoma with parotid and neck lymph node metastases. Complete spontaneous regression of metastatic melanoma is very rare, with an estimated incidence of 0.22%-0.27%. We review the literature on this subject. Elucidation of the process of spontaneous regression may offer the possibility of improved methods of treating and preventing cancer.
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5/11. Regression of fundic gland polyps following acquisition of helicobacter pylori.

    The prevalence of helicobacter pylori infection is very low in patients with fundic gland polyps (FGPs) of the stomach. We report here two cases with multiple FGPs that regressed following new H pylori acquisition. Patient Nos I and II had multiple FGPs in normal fundic mucosa without inflammatory changes or atrophy. Both were not infected with H pylori. Following acquisition of H pylori infection however, all FGPs in both patients completely disappeared except for one FGP in patient No I. Although the size of the remaining polyp in patient No I was greatly reduced after H pylori acquisition, it became enlarged again after eradication. Interestingly, in the remaining polyp, we found an activating beta-catenin gene mutation whereas no such mutations were detected in FGPs of patient No II. Thus H pylori infection may have an inhibitory effect on the development of FGPs.
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6/11. Spontaneous regression of intraoral mucosa-associated lymphoid tissue lymphoma: molecular study of a case.

    Mucosa-associated lymphoid tissue (MALT) lymphoma presentation in the oral cavity is very rare. Reported herein is a case of intraoral MALT lymphoma of the minor salivary gland in a 70-year-old woman with sjogren's syndrome. Unexpectedly, a spontaneous clinically and histologically confirmed regression occurred 1 month after the tumor biopsy for diagnosis. Considering that salivary MALT lymphoma is associated with sjogren's syndrome and that the chronic inflammation caused by sjogren's syndrome persisted, it is hypothesized that the tumor clone might be present in the regressed lesion. Minimal residual tumor clone identical with the primary lesion was detected using the polymerase chain reaction (PCR) clonality assay for immunoglobulin heavy chain gene (IgH) rearrangement. No recurrence was clinically evident 38 months after the diagnosis. Spontaneous regression of MALT lymphoma should be examined at the molecular level in addition to clinical and histological evaluations. When minimal residual disease is detected, close follow up is necessary for early detection of the tumor relapse.
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7/11. Spontaneous regression of squamous cell lung carcinoma with adrenal metastasis.

    A 61-year-old man was found to have squamous cell carcinoma of the left hilum with metastasis to the left adrenal gland documented by needle aspiration. About two years later, the primary tumor is not detectable, and the adrenal gland is of normal size on follow-up computerized tomography. To our knowledge, this is the first documented case of spontaneous regression of squamous cell carcinoma of the lung with adrenal metastasis.
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8/11. Spontaneous regression of pulmonary leiomyomas during pregnancy.

    Multiple pulmonary nodular densities simulating metastastic cancer were discovered in a routine chest roentgenogram of a 30-year-old pregnant woman. lung biopsy revealed nodules composed of smooth muscle and collagenous tissue containing entrapped glandular elements. The lesions were initially interpreted as multiple pulmonary fibroleiomyomatous hamartomas (MPFLH). During pregnancy and the post-partum period, the pulmonary nodules regressed spontaneously. Critical analysis of the published cases as well as our own case indicates that multiple pulmonary fibroleiomyomatous hamartomas (MPFLH) cannot be distinguished from benign metastasizing leiomyoma (BML) by either clinical, roentgenographic, or pathologic criteria and that all represent pulmonary metastases from a primary uterine neoplasm. The spontaneous regression of the pulmonary nodules in the present case as well as the increased risk for development of progressive pulmonary insufficiency in the pre-menopausal patients indicates an apparent hormonal dependence. Total abdominal hysterectomy and bilateral salpingo-oophorectomy appears to be the treatment of choice.
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9/11. neuroblastoma in situ.

    Two cases of neuroblastoma in situ incidentally found in young infants at autopsy are reported. One was an 8-day-old female infant of large for dates clinically diagnosed as fetal erythroblastosis. Histologically, several foci of tumor were scattered within the medulla of the left adrenal gland. This case was thought to be multiple neuroblastoma in situ. The other was a 34-day-old male infant who was born premature and associated with patent ductus arteriosus (PDA). Grossly, a solitary small nodule was seen in the right adrenal gland. Microscopically, the lesion was located within the medulla. In both cases, the tumor was composed of lymphocyte-like small and dark cells with rosette formations. Local infiltrations were observed, but not metastasis. The review of 611 autopsy cases of neuroblastoma reported in the Annual of the Pathological autopsy Cases in japan, vol. 11 (1968) to vol. 20 (1977)2 revealed 7 cases of neuroblastoma in situ including one of our cases. The peculiar features of neuroblastoma were described.
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10/11. Spontaneous disappearance of fundic gland polyposis: report of three cases.

    Three cases are reported in which multiple sessile polyps of the stomach disappeared spontaneously during the follow-up period from 9 to 34 mo. All of the patients were middle-aged females, and the distribution of the polyps was limited to the portion of the stomach with fundic glands. Histolgoic examination revealed simple hyperplasia of the fundic glands with microcysts. The macroscopic and histologic pictures were the same as the gastric lesions ("fundic gland polyposis") found in cases with familial adenomatosis coli; however, colonic polyposis was not observed in our cases. The possible cause of the spontaneous disappearance of the polyps was discussed.
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