OBJECTIVETo study the clinicopathologic features of granulocytic sarcoma.

METHODSThe clinical and pathologic findings of 38 cases of granulocytic sarcoma were retrospectively analyzed. Immunohistochemical study was performed and the literature was reviewed.

RESULTSThe age of patients ranged from 2 to 77 years (mean = 43.3 years). The male-to-female ratio was 1.5:1. Major clinical presentations included superficial lymph node enlargement and painful soft tissue mass. Follow-up data were available in 18 patients; and 14 of them died of tumor-related diseases. The average duration of survival of the patients was 16.9 months. Histologically, the tumor cells were relatively uniform in appearance and small to medium in size. The cytoplasm was scanty and pale in color. The nuclei were round or focally irregular, with fine chromatin and inconspicuous nucleoli. Mitosis figures were readily identified. Scattered immature eosinophilic myelocytes were seen. Immunohistochemical study showed that the tumor cells in all cases expressed MPO and CD43. Most cases were also positive for CD68, lysozyme, CD99 and TdT. The staining for CD3, CD20, CD79a, pan-cytokeratin and PLAP were negative.

CONCLUSIONSGranulocytic sarcoma is a known histologic mimicker of non-Hodgkin lymphoma, Ewing sarcoma/PNET and embryonal rhabdomyosarcoma. Detailed morphologic examination, when coupled with immunohistochemical study, is useful in arriving at a correct diagnosis.

Adolescent ; Adult ; Aged ; Burkitt Lymphoma ; metabolism ; pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Leukosialin ; metabolism ; Lymph Nodes ; pathology ; Male ; Middle Aged ; Muscle Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Ovarian Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Peroxidase ; metabolism ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; metabolism ; pathology ; Retrospective Studies ; Sarcoma, Ewing ; metabolism ; pathology ; Sarcoma, Myeloid ; drug therapy ; metabolism ; pathology ; surgery ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Survival Rate ; Young Adult

The distinction of a spitz nevus from a melanoma can be difficult and in some cases, impossible. A misdiagnosed spitz nevus can metastasize and lead to fatal outcomes, especially in children. A 5-yr-old girl presented with a 1-yr history of a solitary pinkish nodule on her left hand. On physical examination, she had a palpable left axillary lymph node. We performed biopsy and checked 3 sentinel lymph nodes (SLN) on her axillary area. The biopsy specimen showed multiple variably sized and shaped nests with large spindle or polygonal cells and SLN biopsy showed 3 of 3 lymph nodes that were metastasized. Under the diagnosis of spitzoid melanoma, she was treated with excision biopsy and complete left axillary lymph nodes were dissected. She received interferon-alpha2b subcutaneously at a dose of 8 MIU per day, 3 times weekly for 12 months, and shows no recurrence.
Antineoplastic Agents/therapeutic use ; Child, Preschool ; Female ; Humans ; Interferon-alpha/therapeutic use ; Lymphatic Metastasis ; Melanoma/drug therapy/*pathology/surgery ; *Nevus, Epithelioid and Spindle Cell ; Recombinant Proteins/therapeutic use ; Skin Neoplasms/drug therapy/*pathology/surgery

CD4 Antigens ; metabolism ; CD56 Antigen ; metabolism ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Hematologic Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Leukemia, Myeloid ; pathology ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Skin Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery

BACKGROUND AND OBJECTIVEAmyloid deposition is rare. If there was a great amount of amyloid depositions in the skin tissue, it would be considered to be amyloid deposition disease at first, and then primary cutaneous marginal zone B-cell lymphoma (PCMZL). This study was to analyze the diagnosis and differential diagnosis of two cases of PCMZL with amyloid deposition.

METHODSClinicopathologic characteristics and follow-up of two cases of PCMZL were analyzed. Immunohistochemical staining was performed by EnVision method using antibodies LCA, CD19, CD20, CD79a, CD3, CD7, MUM1, kappa, lambda, Ki-67. IgH and TCRgamma gene rearrangement was detected by polymerase chain reactive (PCR).

RESULTSCase 1, a 71-year-old Chinese male, had a subcutaneous mass on the right elbow that was initially diagnosed with "amyloidosis" in 2004. Three years after the initial diagnosis, he developed recurrences on the right para-auxillary that was still diagnosed with "probably amyloidosis". Four years after the first diagnosis, the patient presented a lesion on the right para-auxillary with a diameter of 2 cm and a lesion on the temporal-parietal dural with a size of 6.0 cmx3.0 cmx3.0 cm. Case 2, a 68-year-old Chinese male, had a subcutaneous mass next to back of the left ear with a size of 9.0 cmx5.0 cm, and he underwent a operation one year previously because of subcutaneous mass in the same site. Microscopically, the tumors of both cases were located in dermis and subcutaneous, tumor cells were medium size with a nodular or diffuse distribution, and some of tumor cells were plasmacytoid/plasma cells. Morphologically, the temporal-parietal dural lesion was similar to subcutaneous lesion and infiltrated into cranial (case 1). Juxtaposed the tumor cells of two cases, there were the large amyloid deposits of amorphous hyaline material and concentrically laminated hyaline spherules in case 1, while cord-like amyloid deposits in case 2. Reactive lymphoid follicles with germinal centers and foreign body giant cells in the stroma were found surrounding the amyloid deposits. Congo red staining showed positive of amyloid deposition in tumor tissues of both cases. Immunohistochemical staining revealed that LCA, CD19, CD20, CD79a and MUM1 expressions were positive in tumor cells, and Ki-67 expression was about 8%-10%. IgL restricted expression as kappa positive while lambda negative was found in both cases. PCR results showed monoclone gene rearrangement of IgH gene in both cases.

CONCLUSIONSOur findings suggest that amyloid deposition rarely present in both primary and metastatic tumors in PCMZL, and its diagnosis should be considered to avoid misdiagnosis. The patients with PCMZL should undergo regular examinations and chemotherapy as well as a long-term follow-up since it is apt to recur or relapse.

Aged ; Amyloidosis ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Antigens, CD ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Elbow ; Follow-Up Studies ; Head and Neck Neoplasms ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Interferon Regulatory Factors ; metabolism ; Leukocyte Common Antigens ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Neoplasm Recurrence, Local ; Prednisone ; therapeutic use ; Skin Neoplasms ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Vincristine ; therapeutic use

OBJECTIVETo study the morphologic features, immunophenotypes, differential diagnoses and prognosis of histiocytic sarcoma (HS).

METHODSThe clinical and pathologic findings of 6 cases of HS were reviewed. Immunohistochemical assay (Elivision staining) was also performed. Follow-up information was available in 4 patients.

RESULTSThere were altogether 3 males and 3 females. The age of patients ranged from 12 to 81 years old (median = 54.6 years). The sites of involvement included lymph node (number = 2 cases) and skin or soft tissue (number = 4 cases). The tumor was composed of sheets of large epithelioid cells with abundant eosinophilic cytoplasm, oval to irregular nuclei, vesicular chromatin and large nucleoli. Binucleated form was not uncommon. Two of the cases showed increased pleomorphism with multinucleated tumor giant cell formation. Focal cytoplasmic with foamy appearance was identified in 3 cases. One case demonstrated foci of spindly sarcomatoid appearance. Hemophagocytosis was identified in 2 cases. Mitotic figures were readily identified. The tumor cells were often accompanied by various numbers of inflammatory cells. Immunohistochemical study showed that all cases were diffusely positive for leukocyte common antigen, CD4, CD68 and CD163. Four of the 5 cases studied also expressed lysozyme. Amongst the 4 patients with follow-up information available, 3 died of the disease at 6 to 11 months interval after diagnosis. One patient, whose lesion was localized at the skin and soft tissue, survived for 3 years, with no evidence of tumor recurrence.

CONCLUSIONSAccurate diagnosis of the HS is based on the combination of morphologic examination and immunohistochemical assay. HS often presents with clinically advanced disease and pursues an aggressive clinical course, with a poor response to therapy. However, a subset of cases presenting with clinically localized lesion may carry a relatively favorable long-term outcome.

Adult ; Aged ; Aged, 80 and over ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Child ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytic Sarcoma ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Melanoma ; metabolism ; pathology ; Muramidase ; metabolism ; Prognosis ; Receptors, Cell Surface ; metabolism ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Soft Tissue Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Young Adult