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PubMedDate not supplied

[Bilateral "crocodile tears syndrome" associated with Melkersson-Rosenthal syndrome--case report].

We present a rare case of bilateral crocodile tears syndrome (CTS) in the course of Melkersson-Rosenthal syndrome. Melkersson-Rosenthal syndrome is characterised by a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissured tongue. The classic triad is infrequent and oligosymptomatic variants are seen more frequently. CTS is a rare complication of facial nerve paralysis characterised by inappropriate lacrimation on the side of the palsy in response to salivary stimuli. It results from aberrant reinnervation of the lacrimal gland by salivary parasympathetic fibres. The therapeutic approach for an acute bout of Melkersson-Rosenthal syndrome consists mainly of steroid administration. CTS management is composed of anticholinergic drugs and surgical procedures. Botulin toxin injection into the lacrimal gland is the most modern therapeutic option. In the case presented CTS developed in a 50-year-old man after 5 incidents of facial palsy due to Melkersson-Rosenthal syndrome. The case deserves attention due to the rarity of the observed symptoms and signs.

Botulinum Toxins↗

PubMed1981

Congenital absence of the pulmonary valve associated with ventricular septal defect.

The authors describe 5 cases of an unusual variant of tetralogy of Fallot with a characteristic clinical course. Its most prominent clinical manifestations are recurrent and severe respiratory infections caused by compression of the inferior trachea and bronchi by a dilated pulmonary artery. Two of the patients died of irreversible respiratory insufficiency. Of the 3 surviving patients one is doing comparatively well.

Electrocardiography↗

PubMed1997

Excimer laser phototherapeutic keratectomy: long-term results and practical considerations.

PURPOSE: Our purpose was to evaluate the safety and short- and long-term efficacy of excimer laser phototherapeutic keratectomy (PTK) to treat anterior corneal pathology. METHODS: Twenty-eight eyes of 24 patients underwent PTK for anterior corneal pathologic conditions with a VISX excimer laser. Preoperative indications included granular dystrophy (six eyes), superficial variant of granular dystrophy (six eyes), and Salzmann's nodular degeneration (five eyes). Three eyes had recurrent erosions, and two eyes had keratoconus nodules. Additional diagnoses included other corneal dystrophies and various corneal scars. Mean follow-up time was 22.5 months (range, 5-44). One eye underwent a repeated PTK for recurrent scarring 1 year after the initial procedure and is included in the results. RESULTS: The mean time to reepithelialization was 4.2 days (range, 2-11). Corneal smoothness and clarity improved in all patients. Uncorrected visual acuity improved a mean of 1.4 lines (range, -6 to +8 lines) and best corrected visual acuity improved a mean of 2.7 lines (range, -3 to +7 lines). Mean corneal flattening was 2.13 D (range, 7.75. D flatter to 6.5 D steeper). The preoperative goal was achieved in 22 (78.5%) of 28 eyes, with no significant change in five (18%) eyes and functional worsening in one (3.5%) eye. Five eyes developed moderate to significant recurrences of their pathology, including the retreated eye. CONCLUSION: Excimer laser PTK is safe and effective for the treatment of anterior corneal pathology. Recurrence of pathology, especially corneal dystrophies, do occur with time.

Adolescent↗

PubMed1996

Liposarcoma: outcome and prognostic factors following conservation surgery and radiation therapy.

PURPOSE: Liposarcoma is among the more common types of soft-tissue sarcoma. This retrospective review was performed to determine the outcome and delineate prognostic factors for this disease when treated by conservation surgery and radiation. METHODS AND MATERIALS: Between 1964 and 1992, 112 consecutive patients with liposarcoma were treated with conservation surgery and radiotherapy. Univariate and multivariate statistics were used to evaluate the outcome with local control, metastatic relapse, and survival as end points, and to delineate independently significant prognostic factors. RESULTS: Preoperative radiation to a mean dose of 50 Gy was used in 36 patients and postoperative radiation to a mean dose of 61 Gy was used in 76 patients. At a median follow-up of 9 years (range 2-25), 34 patients (30%) developed disease progression at some site. Local recurrence developed in 14 patients (13%), and the actuarial local relapse rate was 13% at 10 years. Metastases developed in 23 patients (21%) with an actuarial metastatic rate of 23% at 10 years. Five-, 10-, and 15-year survival rates were 79%, 69%, and 61%, respectively. The most important factor influencing outcome--whether local control, metastases, or survival--was tumor histologic subtype. Well-differentiated (15 patients) and myxoid (including round cell) (71 patients) subtypes had a local control rate exceeding 90% at 10 years. No patient with well-differentiated liposarcoma developed metastases. The actuarial metastatic rate for myxoid tumors was 22% at 10 years and was higher for large than small tumors. The pattern of metastases for this variant was atypical with a preponderance of extrapulmonary sites. Pleomorphic liposarcoma (26 patients) was a highly malignant tumor with a high local recurrence rate of 37% and a metastatic rate of 41% at 10 years. Larger pleomorphic tumors had a significantly higher metastatic rate than smaller ones. These biologic factors were reflected in progressively decreasing 10-year survival rates from 87% for well-differentiated lesions, through 76% for myxoid tumors, to 39% for pleomorphic tumors. Although other factors such as positive resection margins and prior local recurrence were also independently significant determinants of outcome, histology was the overriding factor. CONCLUSION: Liposarcoma is a highly pleomorphic disease whose outcome is greatly determined by its histologic subtype: so greatly that results reported without histologic stratification are likely meaningless. Conservation surgery and radiation achieve excellent local results and with relatively little metastatic risk for patients with well-differentiated and myxoid liposarcomas. The same treatment strategy appears appropriate for pleomorphic liposarcoma, but with a much smaller expectation of a satisfactory outcome. The development of effective systemic therapy is an important goal for this variant of liposarcoma.

Adolescent↗

PubMed1991

The symptomatic os subfibulare. Avulsion fracture of the fibula associated with recurrent instability of the ankle.

Four adults who had symptomatic instability of the ankle had an associated os subfibulare. Operative exploration revealed the ossicle to represent a non-union of an avulsion fracture of the anterior talofibular ligament. These findings suggest that an os subfibulare represents an avulsion fracture that may or may not be associated with laxity of the anterior talofibular ligament, rather than being a normal variant.

Adult↗

PubMed2004

Invasive micropapillary carcinoma of the breast: clinicopathologic study of 62 cases of a poorly recognized variant with highly aggressive behavior.

We report 62 cases of invasive micropapillary carcinoma of the breast characterized by delicate pseudopapillary structures lacking a fibrovascular core and by tubuloalveolar structures freely floating in clear, empty spaces. All patients but 1 were women (median age, 57 years; range, 25-89 years). Tumor size ranged from 0.7 to 10 cm (median, 2.8 cm); 54 (87%) were grade 3. Psammoma bodies were identified in 29 (47%). Focal to massive lymphatic permeation was present in 39 (63%). Architectural features were retained in the node metastases, dermal lymphatics, and recurrences. Fifty-six patients (90%) had metastatic axillary nodes: 18 tumors were estrogen receptor-positive (32%); 11 were progesterone receptor-positive (20%); HER2/neu was overexpressed in 53 (95%) and p53 in 39 (70%). A peculiar immunoreactivity for MUC1 limited to the cytoplasmic membrane oriented toward the stroma and an absence of immunoreactivity for E-cadherin in the same side of the cytoplasmic membrane indicated inversion of cell polarization and a disturbance in the cell adhesion molecules. Of 41 patients with available follow-up, 29 (71%) had local recurrence (mean, 30 months) and 20 (49%) died of disease. These results underscore the aggressive behavior and poor prognosis of this breast carcinoma variant. Aggressive preoperative neoadjuvant chemotherapy should be considered.

Adult↗

PubMed2005

Purging disorder: an ominous variant of bulimia nervosa?

OBJECTIVE: The current study evaluates the clinical significance and distinctiveness of purging disorder (PD), an eating disorder characterized by recurrent purging in the absence of objective binge episodes (OBE) among normal-weight individuals. METHOD: Women with PD (n = 37), bulimia nervosa (BN; n = 39), or no eating disorder (n = 35) completed clinical assessments. Women with PD (n = 23) and BN (n = 25) completed 6-month follow-up assessments. RESULTS: Compared with controls, both eating-disordered groups reported significantly higher eating, Axis I, and Axis II pathology. Compared with BN, PD was associated with significantly lower eating concerns, disinhibition, and hunger. At 6-month follow-up, rates of remission did not differ significantly between PD and BN, and crossover between disorders was rare. CONCLUSION: PD appears to be a clinically significant and potentially distinctive eating disorder.

Adolescent↗

PubMed2001

A superfamily of variant genes encoded in the subtelomeric region of Plasmodium vivax.

The malarial parasite Plasmodium vivax causes disease in humans, including chronic infections and recurrent relapses, but the course of infection is rarely fatal, unlike that caused by Plasmodium falciparum. To investigate differences in pathogenicity between P. vivax and P. falciparum, we have compared the subtelomeric domains in the DNA of these parasites. In P. falciparum, subtelomeric domains are conserved and contain ordered arrays of members of multigene families, such as var, rif and stevor, encoding virulence determinants of cytoadhesion and antigenic variation. Here we identify, through the analysis of a continuous 155,711-base-pair sequence of a P. vivax chromosome end, a multigene family called vir, which is specific to P. vivax. The vir genes are present at about 600-1,000 copies per haploid genome and encode proteins that are immunovariant in natural infections, indicating that they may have a functional role in establishing chronic infection through antigenic variation.

Adult↗

PubMed1994

Clinicopathologic analysis of 615 cases of meningioma with special reference to recurrence.

A total of 615 cases of meningioma operated on from 1959 to 1992 at National Taiwan University Hospital were reviewed. Factors influencing tumor recurrence were also evaluated. Of the 566 patients with intracranial meningioma, 350 were females and 216 were males (F:M = 1.6:1). The mean age was 47.5 years. The distribution of histologic type was: 232 meningothelial (41%), 141 transitional (24.9%), 106 fibroblastic (18.7%), 42 atypical (7.4%), 16 anaplastic (2.8%), and 29 angioblastic [5.2%, including five hemangioblastic (0.9%) and 24 hemangiopericytic (4.3%)]. Anatomical locations were parasagittal/falx area (35%), cerebral convexity (19.3%), sphenoid wing (10.2%), posterior fossa (7.4%), tuberculum sellae (6.7%), olfactory groove (5.7%), tentorium (4.8%), middle fossa (3.7%), and others (7.4%). Recurrence occurred in 83 patients, with a total 19.4% recurrence rate at five years. Sex, histologic grade, and tumor location were associated with recurrence. The recurrence rate was 19% for males and 12% for females (p < 0.05); while it was 42.9%, 64.3%, and 69.6%, respectively for atypical, anaplastic and hemangiopericytic meningiomas, which was much higher than 12.2% for benign ones (p < 0.005). Tumors in less accessible surgical sites also had a higher recurrence rate (p < 0.005). There was no correlation between the age of patients, the tumor size, and the histological subtypes and variants. Of the 49 spinal meningiomas, the majority occurred in adult females (80%), were benign in nature (98%) and thoracic in location (71.4%).

Adolescent↗

PubMed2004

Pharmacogenetics of selective serotonin reuptake inhibitor response: a 6-month follow-up.

BACKGROUND: We previously reported the association between some genetic factors and short-term antidepressant outcome. In the present paper we investigated the same gene variants in a prospective 6-months naturalistic follow-up. METHODS: The sample included 185 inpatients affected by recurrent major depression consecutively admitted to the Psychiatric Inpatient Unit of San Raffaele Hospital from 1998 to 2003 and prospectively followed for 6 months after their recovery. All the patients were undertaking continuation therapy. The functional polymorphism in the upstream regulatory region of the serotonin transporter gene (SERTPR), the tryptophan hydroxylase A218C substitution, a VNTR polymorphism located 1.2 kb upstream of the monoamine oxidase-A coding sequences, the CLOCK gene T3111C and the PER3exon15 gene T1940G substitutions were analysed, using PCR-based techniques. RESULTS: No association was found between clinical variables and relapses; subjects showing TT genotype at CLOCK gene tended to relapse within 6 months after recovery more than TC and CC subjects taken together. A non-significant tendency of SERTPR*s/s subjects to a minor frequency of relapse was also observed. CONCLUSION: Some subjects showing remission after acute treatment relapsed within 6 months, despite undertaking a maintenance treatment; the causes could be heterogeneous, but CLOCK gene variants may influence the outcome.

Adult↗

PubMed2001

The morphologic spectrum of hibernoma: a clinicopathologic study of 170 cases.

Hibernoma, an uncommon tumor of brown fat, has been described only in a few case reports and small series. The authors reviewed 170 cases of hibernoma and evaluated the morphologic features and the behavior of this tumor. The records from the Soft Tissue Registry of the Armed Forces Institute of Pathology from 1970 were searched for cases coded as "hibernoma." Clinical information and available slides from 170 hibernomas were reviewed. Immunohistochemical staining for S-100 and CD34 was performed on select cases. Follow-up information was obtained from the patients' medical records, the patients' physicians, and the patients themselves. Of 170 patients with hibernoma, 99 were men and 71 were women. The tumor occurred most commonly in adults, with a mean age of 38.0 years (age range, 2-75 years). Nine tumors occurred in pediatric patients. The most common anatomic locations included the thigh (n = 50), shoulder (n = 20), back (n = 17), neck (n = 16), chest (n = 11), arm (n = 11), and abdominal cavity/retroperitoneum (n = 10). The average duration of the tumor was 30.6 months. Tumor size ranged from 1 to 24 cm with an average dimension of 9.3 cm. All tumors were composed partly or principally of coarsely multivacuolated fat cells with small, central nuclei and no atypia. Four morphologic variants of hibernoma were identified: typical, myxoid, spindle cell, and lipoma-like. "Typical" hibernoma (n = 140) included eosinophilic cell, pale cell, and mixed cell types based on the tinctorial quality of the hibernoma cells. The myxoid variant (n = 14) contained a loose basophilic matrix. Spindle cell hibernoma (n = 4) had features of spindle cell lipoma and hibernoma; all occurred in the neck or scalp. The lipoma-like variant (n = 12) contained only scattered hibernoma cells. Immunohistochemically, 17 of 20 cases (85%) were positive for S-100 protein. Only one hibernoma of 20, a spindle cell variant, was positive for CD34, whereas other hibernoma variants were negative. Follow-up was obtained for 66 cases (39%) over a mean period of 7.7 years (range, 6 months-28 years). None of the patients with follow-up had a recurrence or metastasis, including eight with intramuscular tumors. No patient died of disease. Hibernoma is a tumor found most often in adults and most commonly in the thigh, with several morphologic variants. It is a benign tumor that does not recur with complete excision. Hibernomas should not be confused with atypical lipomas or well-differentiated liposarcoma.

Adolescent↗

PubMed2005

Angiogenesis as an indicator of metastatic potential in papillary thyroid carcinoma.

UNLABELLED: Angiogenesis is new blood vessel formation, a process that can lead to tumor development. Microvessel count has been correlated to metastasis in some neoplasias. PURPOSE: To determine if measurement of microvessel density is useful in predicting metastasis to the cervical lymph node and prognosis in patients with papillary thyroid carcinoma. METHODS: A retrospective analysis was performed in 30 patients that had undergone total thyroidectomy. They were divided in 2 groups of 15 patients--with and without metastatic disease. Immunohistochemistry was used to detect expression of CD34 in archival paraffin-embedded papillary thyroid tumors, and microvessel density was calculated based on it. Association between microvessel density and the presence of metastasis, according to histological subtype, disease recurrence, and AMES prognostic index groups was determined through statistical analysis. RESULTS: The median microvessel density for the patient group without metastasis (200.0 microvessels/mm2) was apparently, but not significantly, less than that observed among metastatic disease patients (254.4 microvessels/mm2) (P=.20). When papillary carcinoma subtypes were analyzed, this difference became significant (P=02). The follicular variant exhibited a greater microvessel density than the other subtypes, independent of metastasis presence. There was an apparent, but not significant, tendency for a larger median microvessel density in the group of patients that presented recurrence (294.4 microvessels/mm2 vs 249.6 microvessels/mm2, P=.11). There was no relationship between risk level and microvessel density: in the low- and high-risk groups, the median MVD was 304.0 microvessels/mm2 and 229.6 microvessels/mm2, respectively (P=.27). CONCLUSIONS: The results suggest that angiogenesis is more intense among metastatic tumors in the classic and the tall cell variants, indicating that microvessel count can be an indicator of the potential for metastasis in these subtypes of papillary thyroid carcinoma. Patients that developed recurrent disease had a tendency to exhibit higher angiogenesis; however, there was no association between microvessel density and the AMES prognostic index.

Adolescent↗

PubMed2002

Interphase FISH assays for the detection of translocations with breakpoints in immunoglobulin light chain loci.

Many B-cell malignancies bear chromosomal translocations juxtaposing immunoglobulin (IG) genes with oncogenes, resulting in deregulated expression of the latter. Translocations affecting the IG heavy chain (IGH) locus in chromosomal region 14q32 are most prevalent. However, variant translocations involving the IG kappa (IGK) locus in 2p12 or the IG lambda (IGL) locus in 22q11 occur recurrently in B-cell neoplasias. No routine methods for the detection of all breakpoints involving IG light chain loci independently of the translocation partner have been described. For this reason, we have designed 2 novel interphase fluorescence in situ hybridization (FISH) assays using differentially labeled probes flanking the IGK and IGL locus, respectively. Based on extensive control studies, the diagnostic thresholds for the detection of breakpoints were set at 0.3% for IGK and 1.4% for IGL. Fifteen cases of B-cell malignancies with cytogenetically detectable chromosomal abnormalities in 2p11-14 were investigated with the FISH assay for IGK. Breakpoints affecting the IGK locus were detected in 7 cases including all 4 variant Burkitt's translocations t(2;8)(p12;q24) and a variant BCL2-associated translocation t(2;18)(p12;q21). Other translocation partners were chromosome bands 7q21 and 16q24. Ten cases with abnormalities in 22q11-12 were investigated with the FISH assay for IGL. Breakpoints in the IGL locus were diagnosed in 7 cases including both variant Burkitt's translocations t(8;22)(q24;q11) and a t(3;22)(q27;q11) involving the BCL6 locus. Other translocation partners were 2p13-14, 4q13 and 16p12. Our results show that these FISH assays provide flexible, simple and reliable tools in the diagnosis and characterization of genetic changes in B-cell malignancies.

Adolescent↗

PubMed1997

Distinct spectrum of CFTR gene mutations in congenital absence of vas deferens.

Congenital absence of the vas deferens (CAVD) is a frequent cause for obstructive azoospermia and accounts for 1%-2% of male infertility. A high incidence of mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene has recently been reported in males with CAVD. We have investigated a cohort of 106 German patients with congenital bilateral or unilateral absence of the vas deferens for mutations in the coding region, flanking intron regions and promotor sequences of the CFTR gene. Of the CAVD patients, 75% carried CFTR mutations or disease-associated CFTR variants, such as the "5T" allele, on both chromosomes. The distribution of mutation genotypes clearly differed from that observed in cystic fibrosis. None of the CAVD patients was homozygous for delta F508 and none was compound heterozygous for delta F508 and a nonsense or frameshift mutation. Instead, homozygosity was found for a few mild missense or splicing mutations, and the majority of CAVD mutations were missense substitutions. Twenty-one German CAVD patients were compound heterozygous for delta F508 and R117H, which was the most frequent CAVD genotype in our study group. Haplotype analysis indicated a common origin for R117H in our population, whereas another frequent CAVD mutation, viz. the "5T allele" was a recurrent mutation on different intragenic haplotypes and multiple ethnic backgrounds. We identified a total of 46 different mutations and variants, of which 15 mutations have not previously been reported. Thirteen novel missense mutations and one unique amino-acid insertion may be confined to the CAVD phenotype. A few splice or missense variants, such as F508C or 1716 G-->A, are proposed here as possible candidate CAVD mutations with an apparently reduced penetrance. Clinical examination of patients with CFTR mutations on both chromosomes revealed elevated sweat chloride concentrations and discrete symptoms of respiratory disease in a subset of patients. Thus, our collaborative study shows that CAVD without renal malformation is a primary genital form of cystic fibrosis in the vast majority of German patients and links the particular expression of clinical symptoms in CAVD with a distinct subset of CFTR mutation genotypes.

Adult↗

PubMed2004

Intronic mutations in the L1CAM gene may cause X-linked hydrocephalus by aberrant splicing.

L1 disease is a clinically heterogeneous X-chromosomal neurodevelopmental disorder that is frequently associated with mental retardation and congenital hydrocephalus in males. It is caused by mutations in L1CAM that encodes a multifunctional transmembrane neuronal cell adhesion molecule. We report our findings on 6 novel intronic L1CAM sequence variants (c.523+5G>A, c.1123+1G>A, c.1547-13delC, c.3323-17dupG, c.3457+3A>T, and c.3457+18C>T), and a recurrent one (c.523+12C>T). While the pathogenic potential of nucleotide changes within the evolutionarily well-conserved splice consensus sequence (c.523+5G>A, c.1123+1G>A, and c.3457+3A>T) is widely accepted, it is not always straight forward to assess the disease relevance of intronic mutations, if they lie outside the consensus. The c.523+12C>T variant co-segregated with X-linked hydrocephalus in two unrelated families. In the mutated allele, a preferentially used novel splice donor site is generated that results in a frame shift due to insertion of the first 10 bp of intron 5 in the mature mRNA, a largely truncated protein, and most likely a functional null allele. The c.1547-13delC mutation creates a new acceptor site resulting in the insertion of 4 additional amino acids at the end of the immunoglobulin like domain 5. In contrast, c.3323-17dupG and c.3457+18C>T seem to be non-pathogenic L1CAM variants.

Base Sequence↗

PubMed2003

Dedifferentiated adamantinoma with revertant mesenchymal phenotype.

In adamantinoma of long bones, an osteofibrous dysplasia-like form with scattered epithelial elements and a classic form with abundant epithelium are distinguished. Osteofibrous dysplasia-like adamantinomas occur in children and adolescents and behave relatively benign, whereas classic adamantinomas predominate in adults and have a more aggressive clinical course. Because some osteofibrous dysplasia-like tumors have progressed to classic adamantinomas, it is hypothesized that the former is a potential precursor of the latter, showing mesenchymal-to-epithelial transformation. We report a new morphologic variant of adamantinoma in three patients with sarcomatoid transformation of the epithelial component: one in a primary tumor and two in local recurrences. One patient died of metastatic disease. Histologically, the tumors showed loss of the original characteristic epithelial differentiation with transition to fields of highly pleomorphic cells without epithelial features, high mitotic count, and deposition of osteoid and chondroid matrix. These dedifferentiated areas showed pankeratin positivity as well, although there were some changes in keratin subclass profile compared with other classic adamantinomas. This peculiar variant of long bone adamantinoma shows that in addition to mesenchymal-to-epithelial transformation in the early stage of development, progression to an aggressive subtype may be associated with epithelial-to-mesenchymal transition ("sarcomatoid dedifferentiation"), in which the epithelial immunophenotype is conserved. Thereby it may serve as an example of the plasticity of the mesenchymal phenotype. When confronted with a biopsy of a cortical tumor of the tibia showing sarcomatoid morphology and keratin positivity, adamantinoma should be included in the differential diagnosis, as its distinction has important implications for treatment and prognosis.

Adolescent↗

PubMedDate not supplied

Intracerebral rhabdoid and papillary meningioma with leptomeningeal spread and rapid clinical progression.

OBJECTIVE AND IMPORTANCE: Rhabdoid meningioma (RM) is a relatively new, Grade III tumor entity according to the latest WHO classification. We report rhabdoid and partly papillary, highly anaplastic, intracerebral meningioma with diffuse leptomeningeal spread and distant SCF metastasis to the cervical cord. CLINICAL PRESENTATION: This 27-year-old female was admitted to the hospital with radiological findings suggestive of a primary brain tumor or a metastasis. After subtotal resection and during radiotherapy, follow-up MRI revealed recurrence, metastasis to meninges at the high cervical level, and diffuse basal leptomeningeal enhancement indicating infiltrating tumor. She died approximately 3 months after onset of symptoms. RESULTS: Histological examination revealed rhabdoid and papillary meningioma with high proliferation rate (80% of MIB1-positive cells), necrosis and extensive brain invasion. It was positive for vimentin and S-100 protein, showed focal epithelial membrane antigen expression and accumulation of intermediate filaments on ultrastructural examination. The recurrent tumor diffusely infiltrated leptomeninges and subarachnoid space. CONCLUSION: This is a rare example of mixed, rhabdoid and papillary variant of meningioma, located entirely within the brain parenchyma and accompanied by a fulminant clinical course. The combination of the histological anaplasia with the highest reported proliferation rate, and loss of the cohesion of neoplastic cells led to diffused infiltration of the leptomeninges and metastasis to the spinal cord.

Adult↗

PubMed1997

Follicular lymphomatoid papulosis.

A case of follicular lymphomatoid papulosis (LyP) is reported. The patient was a 60-year-old Japanese woman. Clinically, cutaneous eruptions were reddish, centrally depressed, dome-shaped papules on the extensor aspect of the forearm. Histologically, they exhibited features that fulfilled the disease criteria described by Pierard, et al., i.e., (Am J Dermatopathol 1980;2:173-80), mixed cellular infiltrates including atypical Reed-Sternberg cell-like type-A cells and mycosis cell-like type-B cells surrounding hyperplastic follicular epithelia. The patient also showed many typical nonfollicular LyP papules, i.e., rhythmically recurrent papules which underwent spontaneous involution within a few weeks, over a 10-year period. The coincidental occurrence of a rare variant of follicular LyP and typical LyP in the same individual further suggests that follicular LyP is merely a histological pattern of LyP involving epithelial adnexae.

Cell Nucleus↗