Cutaneous follicle center lymphoma: a clinicopathologic study of 19 cases.

Cutaneous follicle center lymphoma (FCL) is reported to have a unique immunophenotype and clinical course as compared with nodal FCL. We studied 19 cases of FCL of the skin using paraffin embedded tissue. An immunohistochemistry panel included CD45, CD3, CD20, CD43, CD21, bcl-2, bcl-6, CD5, and CD10. Molecular studies were performed by polymerase chain reaction for immunoglobulin heavy chain (IgH) and t(14;18). Trisomy 3 was performed by fluorescent in situ hybridization (FISH) in 13 cases. Follow up was obtained in 17 cases (range 3 to 137 months). Patients included 10 females and 9 males ranging in age from 33 to 88 years at first presentation (mean, 64). Twelve of 19 presented in the head and neck and 6 in the trunk and 1 on the arm. All had no known lymph node disease at presentation. Seventeen patients had no nodal disease with a minimum 3 month follow-up; 2/19 had unknown lymph node status with no follow-up. All cases were immunoreactive with CD20 and negative with CD3. Bcl-2 was immunoreactive in 11/18 cases, bcl-6 in 15/15, CD10 in 14/17, CD43 in 2/16 (both were CD10 immunoreactive) and CD5 in 1/15 (it was also bcl-6 immunoreactive). Eight of 18 cases were monoclonal for IgH. Three of 17 showed the presence of t(14;18). FISH was positive in 4 cases for trisomy 3 ranging from 16 to 22% (12% threshold). Follow-up showed no evidence of disease in 14/17 patients (4 to 137 mos). 3/17 patients are alive with disease (17 to 100 mo), and no patients died of disease.

Unusual presentation of secondary syphilis in 2 HIV-1 positive patients.

Due to diverse clinical and histopathological presentations, diagnosis of secondary syphilis can occasionally prove challenging. This is especially true in the setting of human immunodeficiency virus (HIV) infection. Variable clinical presentations of secondary syphilis in HIV disease may result in an incorrect diagnosis and an inappropriate treatment regimen. Similarly, the histology of secondary syphilitic lesions may show considerable variation, depending on the clinical morphology of the eruption. We report 2 cases of secondary syphilis in HIV-1-infected patients with cutaneous lesions of variable clinical presentation and an unusual lymphoid infiltrate simulating mycosis fungoides.

Extranodal marginal zone B-cell lymphoma of the skin: a morphologic and immunophenotypic study of 11 cases.

Extranodal marginal zone B-cell lymphoma (MZBL) is a recently recognized low-grade lymphoma that has been well described in other organs such as the stomach and salivary gland. It has only recently been described in skin, where it may be difficult to distinguish from reactive processes and other types of B-cell lymphoma such as follicle center lymphoma. These cases may have been classified as pseudolymphomas in the past. Extranodal MZBL was referred to as mucosa-associated lymphoid tissue (MALT) lymphoma before the Revised European-American Classification of Lymphoid Neoplasms was published in 1994. Important histologic features that aid in the diagnosis of MALT lymphoma are atypical lymphocytes (centrocyte-like and monocytoid B cells) often admixed with plasmacytoid lymphocytes, a prominent plasma cell component, lymphoepithelial lesions, intranuclear inclusions (Dutcher bodies), and reactive germinal centers that may be colonized by neoplastic cells. Immunophenotypic studies demonstrating a B-cell phenotype, light chain restriction, coexpression of CD43, and staining of atypical lymphocytes with bcl-2 support a diagnosis of MALT lymphoma. We studied 11 cases of extranodal MZBL of the skin from the Armed Forces Institute of Pathology files. There were six women and five men ranging in age from 30 to 69 years (median, 54 years). The anatomical sites included the trunk, head and neck areas, and upper extremities. There were no other sites of disease besides the skin in any of the cases. The follow-up period ranged from 5 months to 8 years (median, 24 months). Histologic results included an atypical lymphoid infiltrate with B-cell phenotype, reactive germinal centers, and a variable plasma cell component in all cases. No Dutcher bodies or lymphoepithelial lesions were noted. Extranodal MZBL of skin is a diagnostic challenge because of a heterogeneous cellular infiltrate that may be interpreted as a reactive process. The most significant neoplasm with which it is confused is follicular lymphoma. It is important to recognize the characteristic histologic and immunophenotypic features of extranodal MZBL so that the appropriate therapeutic approach may be applied.

Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma in childhood.

Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma (ALCL) is an unusual tumor in the pediatric population. However, the nodal-based form of the disease compared with other histologic subsets of childhood non-Hodgkin's lymphomas (NHL) more frequently involves skin, soft tissue, and bone. The objective of this article is to determine the histologic and immunologic characteristics of childhood primary cutaneous Ki-1(CD30) positive ALCL and its prognosis. The clinical data, histologic features and immunohistochemical profiles of skin biopsy specimens from 3 children with cutaneous Ki-1(CD30) positive lymphoma were reviewed. A literature search was performed and disclosed information on 5 childhood cases. The 3 patients with primary cutaneous Ki-1(CD30) positive ALCL all presented similarly as rapidly growing masses initially and clinically believed to be infectious/reactive processes. The diagnosis was established on the basis of histopathologic examination and immunohistochemical studies. Histologic sections revealed an extensive infiltrate of tumor cells extending throughout the entire dermis into the subcutaneous fat with frank ulceration in 1 patient. No significant epidermotropism was noted. Tumor cells exhibited striking cellular pleomorphism and a high mitotic rate with numerous atypical mitoses. Inflammatory cells were present in all patients. The tumor cells stained positively for Ki-1 antigen (CD30), epithelial membrane antigen, and for T-cell markers (UCHL-1, CD3). One of 3 cases, however, failed to stain for leukocyte common antigen (LCA). No clinically apparent adenopathy was observed in any of the patients. In all instances the patients developed recurrent disease in the skin at sites separate from the primary location. None of the patients demonstrated any involvement of lymph nodes, bone marrow, or other organ systems. All patients were treated with chemotherapy with good response. Primary cutaneous Ki-1(CD30) positive lymphoma is rare in children and is characterized by recurrences. The prognosis seems to be favorable.

Is CD30 (Ki-1) immunostaining in cutaneous eruptions useful as a marker of Th1 to Th2 cytokine switching and/or as a marker of advanced HIV-1 disease?

CD30 is a member of the tumour necrosis factor/nerve growth factor receptor superfamily, which is expressed on CD4+ and CD8+ T-cell clones which produce T helper (Th) 2-type cytokines. It has been proposed that disease progression in HIV-1 is associated with Th1 to Th2 cytokine switching. In 70 cutaneous biopsies from HIV-1 positive patients in different stages of disease, we performed a battery of immunohistochemical stains. These included antibodies to CD3, UCHL-1, OPD-4, L-26, KP-1 and CD30 (Ki-1). In addition, we used a similar battery of stains on cutaneous biopsies of HIV-1 negative patients with inflammatory dermatoses which are established as Th1 or Th2 dominant, e.g. polar leprosy. CD30+ cells were rarely present in early stages of HIV-1 disease, but commonly present in later stages of disease. However, there were cases of late HIV-1 disease which did not contain CD30+ cells. Increased numbers of CD30+ cells were more commonly seen in later stages of HIV-1 disease. However, the expression of CD30 appeared to be better in predicting other established Th2 cutaneous infiltrates in HIV-1 negative patients than in predicting a Th2 cutaneous cytokine pattern in advanced HIV-1 disease.

Unusual expression of S-100 protein in histiocytic neoplasms.

Twenty-nine cases of histiocytic neoplasms, some resembling juvenile xanthogranuloma (JXG) and others resembling reticulohistiocytoma (RH), were evaluated. Immunohistochemical stains were performed. In this series, seven cases were identified that expressed S-100 protein positive cells. The S-100 positive cells were predominantly large mononuclear and multinucleated histiocytes with eosinophilic cytoplasm, but also in some cases xanthomatous cells and Touton giant cells. These cells also expressed a positive reaction for vimentin, KP-1, and Factor XIIIa. There was no reactivity observed for monoclonal antibody 010(CD1a). A positive reaction for S-100 protein is conventionally accepted as a useful differentiating feature between histiocytosis X and non-X histiocytosis such as JXG and RH. The conflicting results of the immunohistochemical stains in the lesions we studied could be potential pitfalls in diagnosing histiocytic neoplasms.

Atypical fibroxanthoma. An unusual variant with osteoclast-like giant cells.

We report three cases of an unusual variant of atypical fibroxanthoma with numerous multinucleated osteoclast-like giant cells. Histologically, the lesions were composed of uniformly dispersed multinucleated osteoclast-like giant cells within a pleomorphic cellular proliferation with microscopic and histochemical features of atypical fibroxanthoma. Multinucleated osteoclast-like giant cells, however, stained with KP-1, a macrophage-associated antigen only. These cells are not necessarily an integral part of the tumor and may represent a reactive histiocytic component.

A comparison of clinical, morphological and immunohistochemical features of lymphomatoid papulosis and primary cutaneous CD30(Ki-1)-positive anaplastic large cell lymphoma.

The advent of immunoperoxidase technique on paraffin embedded tissue has identified a number of shared immunologic markers present in various lymphoproliferative cutaneous disorders. Two such disorders are the recently described primary cutaneous CD30-positive anaplastic large cell lymphoma (ALCL) and lymphomatoid papulosis; both entities are characterized by CD30-positive large atypical cells predominantly of T cell origin. We have compared the clinical, morphological and immunohistochemical features of 50 patients with lymphomatoid papulosis to a group of 27 patients with cutaneous CD30-positive ALCL. There are clear differences between the clinical presentation in these two diseases, and although both are characterized by similar atypical cells, the histologic pattern and distribution of atypical cells is sufficiently different to allow distinction and specific diagnosis based on hematoxylin and eosin stained sections supported by the immunohistochemical stains. In addition, both diseases are characterized by a long benign course, rarely complicated by development of lymphoreticular malignancy and invariably demonstrate CD30 (Ki-1) antigen positive large atypical cells.

Syringolymphoid hyperplasia with alopecia. A case report.

Syringolymphoid hyperplasia with alopecia is an uncommon skin disorder described in 1969 by Sarkany. Since then only three additional cases have been reported, all in the European literature. We present a 59-year-old man with two persistent hyperpigmented hairless patches of 13 years duration. Biopsy specimens revealed characteristic hyperplastic changes of the eccrine glands and ducts and a dense lymphocytic infiltrate surrounding eccrine structures with "syringotropism". Perifollicular lymphocytic infiltration and changes consistent with follicular mucinosis and mycosis fungoides were also present. Imunophenotyping and gene rearrangement studies showed the cells to be primarily of the T helper phenotype with rearrangement of the surface receptor gene.

Primary cutaneous CD30-positive anaplastic large cell lymphoma. Report of 27 cases.

The clinical, morphological and immunohistochemical features of 27 patients with anaplastic large cell lymphoma (ALCL) of CD30-positive type, with cutaneous lesions as the sole initial clinical manifestation, were analyzed. The neoplasm presented as solitary or multiple, usually ulcerated skin lesions, affecting predominantly elderly patients (median age: 67 years) with a male preponderance (male to female ratio of 6:1). In most patients, there was an excellent response to chemotherapy. The cardinal histological features included diffuse dermal and subcutaneous infiltration by large, anaplastic tumor cells, all or nearly all of which showed diffuse, strong membrane staining and frequently a paranuclear, dot-like reaction with the CD30 marker (Ber-H2). Epidermal ulceration, pseudo-epitheliomatous hyperplasia and dermal vascular proliferation were also observed.

Occurrence of multiple myeloma three years after successful renal transplantation.

A living-related renal transplant recipient developed premature progressive allograft dysfunction 3 years after transplantation. Histopathologic examination of a transplant biopsy specimen demonstrated kappa light chain nephropathy, whereupon the diagnosis of multiple myeloma was subsequently confirmed. A variety of tumors have been reported to develop in the successful renal allograft recipient, although multiple myeloma is decidedly uncommon. This may be due to the relatively young age of transplant recipients. Histopathologic examination of renal tissue in the evaluation of premature or unexpected renal failure in the transplant recipient is underscored.

Leiomyosarcoma of ligamentum teres of liver: case report.

Presented is a leiomyosarcoma of the ligamentum teres of the liver in a 10-year-old girl. This is a second such case to be recorded in the literature of the juvenile patient and the first to be studied by electron microscopy.

Heterotopic brain presenting as a cystic neck mass with mandibular deformity.

A congenital cystic neck mass causing respiratory distress and hypoplasia of the mandible was excised from a 3-month-old infant. The lesion was subsequently diagnosed as heterotopic brain, a rare, benign condition that should be considered in the differential diagnosis of the neonatal head and neck mass.

Goodpasture's syndrome--local and diffuse deposition of antibody in glomerular basement membrane. Case report.

An unusual case of Goodpasture's syndrome in a 22 year old white female is presented. The diagnosis of antiglomerular basement membrane disease was made on the basis of circulating anti-GBM antibodies in the presence of linear IgG deposits along the glomerular basement membrane by immunofluorescent microscopy. An additional finding was the presence of discrete granular subepithelial deposits on electron microscopy. Possible pathogenetic mechanisms are discussed.

Prognostic factors in patients undergoing lymphadenectomy for malignant melanoma.

Review of a 19 year experience in melanoma patients undergoinglymphadenectomy at the National Cancer Institute revealedthat the preoperative assessment of the status of theregional lymph nodes was accurate 91% of the time when thesurgeon felt the nodes were clinically positive, and accurate79% of the time when the nodes were judged clinically negative. The 10-year survival in patients with one to three histologicallypositive nodes or no positive nodes was 50-55%, compared to a25% 8-year survival in patients with four or more histologicallypositive nodes. Stepwise multivariate evaluation of prognosticfactors indicated that the most important factor for predictingprognosis is the number of nodes histologically involved. Nodepalpability was the second most important factor because of itshigh correlation with number of nodes histologically involved. Site of melanoma was the third most important factor, aspatients with extremity (upper or lower) melanoma had a bettersurvival (P = 0.002) than patients with axial melanoma (trunkor head and neck). Five years following lymphadenectomythere appeared to be substantial differences in survivalaccording to differences in the level of invasion of the primarylesion, however, these differences were not nearly aspronounced 10 years following node dissection.B The division of melanoma thicknesses into <1.50 mm and>1.50 mm provided some prognostic discrimination at fiveyears but again the differences were not pronounced 10 yearsfollowing node dissection. The thickness measurements wereeasier to determine than the level of invasion, and more reproduceableon resubmission to the same pathologist. Fourpatients with melanoma less than 0.76 mm had subsequentmetastases, but these may represent inadequate sampling of theprimary melanoma both in our series and in the four similarpatients previously reported with such thin metastasizingmelanomas.

Effects of murine tumors on delayed hypersensitivity to dinitrochlorobenzene. I. Description of anergy caused by transplanted tumors.

In the murine model presented for tumor-associated immune suppression, normal BALB/c mice displayed significant foodpad swelling when sensitized on the flank with 2 mg dinitrochlorobenzene (DNCB) dissolved in dimethyl sulfoxide and challenged in a footpad with 0.05 mg DNCB 10 days later. This reaction in challenged footpads seemed to be a classic delayed hypersensitivity reaction, since it took 24 hours to develop and included an extensive mononuclear infiltrate. The reaction was transmissible from sensitized to normal mice by the transfer of lymphoid cells but not to serum. When sensitized 10 days after tumor inoculation, mice bearing either an allogeneic melanoma or a syngeneic lymphoma or fibrosarcoma did not demonstrate delayed hypersensitivity to DNCB.

Malignant melanoma of the skin metastatic to the eye. Frequency in autopsy series.

Results of histopathologic examination of the eyes of 15 consecutive patients with metastatic malignant melanoma arising in the skin demonstrated evidence of intraocular metastasis in five patients. The metastases were microscopic, epithelioid, minimally pigmented, and occurred in both the choroid and the retina. All patients were asymptomatic. Those patients with ocular metastases all had primary malignant neoplasms of the superficial, spreading variety and associated central nervous system metastases.