Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis.

The perivascular epithelioid cell has been proposed to be the unifying proliferating cell type in a number of lesions such as angiomyolipoma, lymphangiomyomatosis, clear cell "sugar" tumor and renal capsuloma. With the exception of rare examples of angiomyolipoma, they are non-metastasizing. We report four examples of a new member of this family of perivascular epithelioid cell neoplasms that occur in abdominopelvic location and show metastatic properties. The patients, all women, were aged 19 to 41 years (mean, 32), and presented with a tumor mass involving the serosa of the ileum, uterus or pelvic cavity. Morphologically, the tumors were composed of sheets of large polygonal cells with glycogen-rich clear or eosinophilic cytoplasm and moderately pleomorphic nuclei, traversed by a delicate vasculature, mimicking clear cell carcinoma. There were areas of coagulative necrosis and occasional mitotic figures. Intracytoplasmic brown pigment was present in two cases. Spindly cells, smooth muscle and fat were absent. Lymphovascular invasion was present in all, lymph node metastasis was documented in two and metastasis to the ovary was present in one case. Two patients developed widespread metastatic disease after 10 and 28 months from diagnosis. One patient showed the clinical signs of tuberous sclerosis. In spite of the epithelial-like appearance, the tumor cells were negative for epithelial markers but were strongly positive with the melanogenesis-related marker HMB45. Another melanogenesis marker (MART-1) was positive in two cases. Other markers including S-100 protein, vimentin, muscle-specific actin, desmin and chromogranin A were negative. Thus, these tumors are not readily classifiable in the existing schema of known entities, and show overlapping morpho-phenotypic features of clear cell "sugar" tumor of the lung and epithelioid angiomyolipoma. We consider them as sarcomas composed of a pure population of uncommitted perivascular epithelioid cell, that lack modulation toward smooth muscle or adipose cells.

T-cell prolymphocytic leukemia with a novel translocation (6;11)(q21;q23).

T-cell prolymphocytic leukemia (T-PLL) is an uncommon chronic lymphoproliferative disorder characterized by lymphadenopathy, splenomegaly, and lymphocytosis. The leukemic cells have the appearance of prolymphocytes and usually an immunophenotype of T-helper cells (CD3+ CD4+ CD8-). Inv(14q), del(11q), i(8q), and rearranged Xq28 are the commonest nonrandom chromosomal abnormalities in T-PLL. Recently, it has been shown that the ataxia-telangiectasia mutated (ATM) gene located at 11q23 is often deleted in T-PLL, suggesting a tumor suppressor role of the ATM gene on tumorigenesis of T-PLL. We report a case of T-PLL with t(6;11)(q21;q23) as the sole chromosomal abnormality and suggest that the cytogenetically identified translocation also implicates the ATM gene.

Nasal pleomorphic adenoma with skeletal muscle differentiation: potential misdiagnosis as rhabdomyosarcoma.

Pleomorphic adenoma can show diverse lines of differentiation in the epithelial and myoepithelial elements, such as cartilage, bone, and fat. Myoid differentiation, however, has not been documented. We report an unusual case of nasal pleomorphic adenoma which shows focal skeletal muscle differentiation. The tumor was discovered only after successful radiation therapy for an undifferentiated carcinoma of the nasopharynx. Apart from being of morphological interest, the presence of cells with skeletal muscle differentiation also raises the practical issue of possible confusion with rhabdomyosarcoma in a biopsy. The distinguishing features are lack of cytological atypia and presence of a neoplastic glandular element.

Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm.

Expression of the natural killer (NK) cell antigen CD56 is uncommon among lymphomas, and those that do are almost exclusively of non-B-cell lineage and show a predilection for the nasal and nasopharyngeal region. This study analyzes 49 cases of nonnasal CD56+ lymphomas, the largest series to date, to characterize the clinicopathologic spectrum of these rare neoplasms. All patients were Chinese. Four categories could be delineated. (1) Nasal-type NK/T cell lymphoma (n = 34) patients were adults 21 to 76 years of age (median, 50 years), including 25 men and 9 women. They presented with extranodal disease, usually in multiple sites. The commonest sites of involvement were skin, upper aerodigestive tract, testis, soft tissue, gastrointestinal tract, and spleen. Only 7 cases (21%) apparently had stage I disease. The neoplastic cells were often pleomorphic, with irregular nuclei and granular chromatin, and angiocentric growth was common. The characteristic immunophenotype was CD2+ CD3/Leu4- CD3epsilon+ CD56+, and 32 cases (94%) harbored Epstein-Barr virus (EBV). Follow-up information was available in 29 cases: 24 died at a median of 3.5 months; 3 were alive with relapse at 5 months to 2.5 years; and 2 were alive and well at 3 and 5 years, respectively. (2) Aggressive NK cell leukemia/lymphoma (n = 5) patients presented with hepatomegaly and blood/marrow involvement, sometimes accompanied by splenomegaly or lymphadenopathy. The neoplastic cells often had round nuclei and azurophilic granules in the pale cytoplasm. All cases exhibited an immunophenotype of CD2+ CD3/Leu4- CD56+ CD16- CD57- and all were EBV+. All of these patients died within 6 weeks. (3) In blastoid NK cell lymphoma (n = 2), the lymphoma cells resembled those of lymphoblastic or myeloid leukemia. One case studied for CD2 was negative and both cases were EBV-. One patient was alive with disease at 10 months and one was a recent case. (4) Other specific lymphoma types with CD56 expression (n = 8) included one case each of hepatosplenic gammadelta T-cell lymphoma and S100 protein+ T-cell lymphoproliferative disease and two cases each of T-chronic lymphocytic/prolymphocytic leukemia, lymphoblastic lymphoma, and true histiocytic lymphoma. All of these cases were EBV-. Six patients died at a median of 6.5 months. Nonnasal CD56+ lymphomas are heterogeneous, but all pursue a highly aggressive clinical course. The nasal-type NK/T-cell lymphoma and aggressive NK cell leukemia/lymphoma show distinctive clinicopathologic features and a very strong association with EBV. Blastoid NK cell lymphoma appears to be a different entity and shows no association with EBV.

T- and T/natural killer-cell lymphomas of the salivary gland: a clinicopathologic, immunohistochemical and molecular study of six cases.

Primary salivary gland lymphomas are almost always of B lineage, with most being represented by low grade B-cell lymphoma of mucosa-associated lymphoid tissue. This study characterizes the rare non-B-cell lymphomas of the salivary gland based on an analysis of six cases. All patients were men, with a mean age of 53.5 years. They presented with submandibular or parotid mass, which on histological examination showed extensive interstitial infiltration by small, medium-sized, or large lymphoid cells. There was prominent invasion and expansion of the ducts and acini in five cases. Angioinvasion was evident in two cases. Three cases were of T lineage and were CD56 negative; one of these cases expressed CD30. Three cases showed an immunophenotype of CD2+ CD3(f)- CD3(p)+ CD56+, consistent with T/natural killer (NK) cell lymphoma. In situ hybridization for Epstein-Barr virus (EBV)-encoded early nuclear RNA (EBER) showed positive reaction exclusively in the three CD56+ cases. Clonal T-cell populations were shown in two CD56-negative cases by polymerase chain reaction on paraffin sections using primers for the T-cell-receptor (TCR) gamma-chain gene, but not in the other four cases (the three CD56+ cases and one CD56- case). Four patients (two CD56+ and two CD56-) died within 3 years, and two were disease free at 4 and 1.5 years, respectively. This study shows that salivary gland T- or T/NK-cell lymphomas cannot be reliably distinguished from B-cell lymphomas on morphological grounds alone, because both can show prominent lymphoepithelial lesions. It appears that T/NK-cell lymphomas, which are often extranodal in localization and strongly associated with Epstein-Barr virus (EBV), show a predilection to involve the salivary glands as well.

Sphenoidal sinus mucocoele and yawning after radiation treatment for nasopharyngeal carcinoma.

The long term complications of radiotherapy in treating patients with nasopharyngeal carcinoma (NPC) are well recognized. Among these, neurological and endocrinological complications are usually considered to be more clinically important. On the other hand, postirradiation sinusitis is often neglected or overlooked because symptoms are usually non-specific or not clinically disturbing. This leads to the under-reporting of this complication. We report the case history of a patient with NPC who developed recurrent and debilitating bouts of yawning attacks 13 years after radiotherapy. The attacks were thought to be due to the compression of the hypothalamus by a large mucocoele in the sphenoidal sinus, which was successfully managed by surgical drainage.

T-cell form of chronic lymphocytic leukaemia: a reaffirmation of its existence.

Early in the 1980s three categories of T-cell chronic lymphocytic leukaemia were recognized: CD4+ CD8- knobby type, CD4- CD8+ azurophilic type and CD4+ CD8- adult T-cell leukaemia (ATL) type. Both azurophilic and ATL types were later shown to be distinctive disorders, whereas the knobby type has been largely neglected and even considered non-existent by some authors. In this report we describe two patients with leukaemia of CD3+ CD4+ CD8- post-thymic T lymphocytes presenting with marked lymphocytosis, generalized lymphadenopathy and hepatosplenomegaly. We believe that CLL of the post-thymic T-lymphocytes is a distinct entity, and merits a separate designation from other T-cell leukaemias.

B-cell small lymphocytic lymphoma with circulating granular prolymphocytes and a novel trisomy 15 anomaly.

A 53-year-old man presented with cervical lymphadenopathy and massive splenomegaly. Peripheral blood examination showed many prolymphocytes with cytoplasmic azurophilic granules, giving an initial impression of large granular lymphocytosis. The lymph node biopsy and immunohistochemical study findings, however, were more compatible with a diagnosis of B-cell small lymphocytic lymphoma. The circulating prolymphocytes showed restricted kappa light chain expression similar to the lymphoid infiltrate in the lymph node. Karyotypic analysis revealed trisomy 15, a chromosomal abnormality that has rarely been described in small lymphocytic lymphoma or chronic lymphocytic leukemia.

Experience with the management of ovarian germ cell tumors in Chinese patients.

Treatment results of 37 consecutive patients with primary ovarian germ cell tumors (OGCTs) were analyzed. Thirty-three were referred after initial laparotomy and four were first seen at relapse. Four patients with stage I dysgerminoma and grade 1 immature teratoma were observed after operation without recurrence. There was also no relapse in eight patients with dysgerminoma given postoperative irradiation (whole abdomen, median 30 Gy). Twenty-five patients (3 dysgerminomas, 11 immature teratomas, 9 endodermal sinus tumors, and 2 mixed germ cell tumors) received short-term cis-platinum-based chemotherapy. Six out of eight measurable tumors treated by chemotherapy had complete remission. Complete follow-up information was obtained in 35 out of 37 patients. The 4-year actuarial survival rates of the whole group and those referred immediately after initial surgery were 94.1 and 100%, respectively. cis-Platinum was substituted by carboplatin in eight cases but this did not affect treatment result. Nonetheless, deaths occurred in two of four patients referred at relapse with extensive disease and initially treated with suboptimal regimens. Chemotherapy-induced side effects were common but mostly tolerable and were related to cis-platinum and bleomycin. The results of this series show that cis-platinum-based chemotherapy is so effective that nearly 100% cure can be achieved in OGCTs and suggest that it is important to institute optimal chemotherapy from the start. On the other hand, common side effects of treatment and possible late toxicities make it desirable for future studies to see whether chemotherapy intensity could be reduced in patients with good prognosis.

Anaplastic large cell Ki-1 lymphoma of bone.

Anaplastic large cell Ki-1 lymphoma is an uncommon type of non-Hodgkin's lymphoma that rarely presents primarily in the bone. Three such cases are reported. All patients were young and had bone pain; one had paraparesis as a complication of collapse of the thoracic vertebral body. The involvement was either monostotic or polyostotic. Radiologically, the lesions were lytic and had ill-defined borders. Histologically, the large neoplastic cells had pleomorphic bizarre nuclei, prominent nucleoli, abundant deeply amphophilic cytoplasm, and paranuclear pale hof. They were admixed with variable numbers of inflammatory cells. One case each was of T-cell, B-cell, and non-T non-B lineage. All three cases showed excellent responses to chemotherapy with or without radiation therapy. Recognizing the lymphomatous nature of this highly pleomorphic tumor is important because of its potential curability with appropriate chemotherapy.

Cutaneous relapse of nasal T-cell lymphoma clinically mimicking erythema multiforme.

Lymphomatous involvement of skin usually manifests as plaques, maculopapules, papulonodules, tumorous masses or ulcerated eruptions. We report an unusual case in which the cutaneous relapse of nasal T-cell lymphoma clinically mimicked erythema multiforme by the abrupt onset of lesions and the presence of targetoid and vesicular lesions. Histologically, the lymphomatous involvement was predominantly periadnexal, with destructive infiltration of blood vessels, nerves and sweat glands. Early biopsy of all unexplained cutaneous eruptions in patients with malignant lymphoma is therefore recommended.