Primary giant cell malignant fibrous histiocytoma of the lung: A case report

A rare case of malignant fibrous histiocytoma of giant cell type originating in the lung of a 46-year-old woman is presented. The patient complained of having a cough that had lasted for a few weeks. A chest X-ray photograph showed a tumor shadow on the left lung. Histological and cytological examination of the biopsy specimen revealed that the tumor was a kind of sarcoma. An operative procedure was selected because of tumor invasion into the trunk of the left pulmonary artery, which was discovered on computed tomography examination, and because metastatic tumor was excluded clinically. The tumor was almost encapsulated and 6 x 6 x 6 cm in size; however, it also showed invasion into the pulmonary artery and bronchial lumen. A histological survey of the tumor showed a wide range of patterns such as fibrous, pleomorphic, fascicular and osteoclast-like giant cell figures; however, the osteoclast-like giant cell area was predominant. Immunohistochemically, the tumor cells were positive for vimentin, CD68 for histiocytic marker and alpha1-antichymotrypsin, and negative for keratin, epithelial membrane antigen, S-100 protein, MT-1, desmin, myoglobin and lysosome. No primary tumor was found clinically in any part of the patient's body at 2 and 4 months after operation. Consequently, she was diagnosed as having primary giant cell malignant fibrous histiocytoma of the lung.     

Malignant giant cell tumor of tendon sheath

Summary A benign, but aggressive, giant cell tumor of tendon sheath developed over a period of 20 years into a metastasizing, histologically malignant giant cell tumor. Ultrastructure of the malignant tumor showed the same five cell types as described in giant cell tumors of tendon sheath. Even the same crystals were identified in the osteoblast-like and osteoclast-like cells.It therefore appears reasonable to assume that giant cell tumors of tendon sheath indeed are neoplasms with a malignant potential and not an inflammatory reaction of synovial cells as previously suggested.Both the benign and malignant tumors seem to be of mesenchymal derivation with partial osseous differentiation. No ultrastructural similarities with fibrous histiocytoma were apparent.     

Significance of giant cells in fine-needle aspiration biopsies of benign and malignant lesions of the pancreas

The cytologic results of 44 consecutive fine-needle aspiration (FNA) biopsies of the pancreas are reported. The series consisted of 27 women and 17 men with an age range of 31-89 yr (mean, 61.5). Excluding insufficient cases, the sensitivity of the procedure was 88%, specificity was 100%, positive predicative value (PV) was 100%, negative PV was 69%, and efficiency of the test was 90%. There were 29 true-positive, four false-negative, and nine true-negative diagnoses. Two specimens were insufficient for diagnosis. Giant cells of varying types were seen in both the malignant and benign cases. Two of the benign cases demonstrated rare multinucleated foreign body-type giant cells, most likely representing the changes seen in pancreatitis. In 13 malignant cases, multinucleated tumor cells were present, while six additional cases had multinucleated benign histiocytes reflecting the associated pancreatitis. Two malignant cases each had tumor giant cells and benign multinucleated histiocytes. Three of the malignant cases had numerous multinucleated tumor giant cells arranged in a dissociative fashion with evidence of cytophagocytosis consistent with a pleomorphic giant-cell carcinoma of the pancreas. One additional case demonstrated numerous multinucleated osteoclastic-like cells consistent with an osteoclastic tumor of the pancreas. This article documents the accuracy of FNA biopsy of the pancreas and notes that giant cells of varying types can be found in pancreatic FNA biopsies. Appreciation of the various types of giant cells in pancreatic FNA biopsy is important for diagnostic accuracy and prognosis.     

Malignant fibrous histiocytoma of the pancreas

An extremely rare case of malignant fibrous histiocytoma In the pancreas Is reported. A 70-year-old man complained of upper abdominal discomfort. A tumor in the head of the pancreas was demonstrated by ultrasonography and computed tomography. The surgical specimen revealed a relatively well demarcated tumor, 9 × 7 × 6.5cm in size. Microscopically, fibroblastic, histiocytic, and muitinucleated giant tumor cells were observed in the myxoid area, but some tumor cells had proliferated in a storiform-pleomorphlc pattern. Immunohtstochemically, some tumor cells were positive for lysozyme, α-1-antitrypsin, α-1-antichymotrypsin, and vimentin. Electron microscopically, tumor cells showed a combination of fibroblastic and histiocytic features. The patient Is currently well with no evidence of recurrence or metastasis 22 months after operation.     

Epithelioid leiomyosarcoma with osteoclast-like giant cells in the rectum

We report a rare case of rectal epithelioid leiomyosarcoma with osteoclast-like giant cells. A 71-year-old Japanese man was admitted to a hospital with melena. Results of a colonoscopy test revealed a polypoid tumor in the rectum, and a biopsy specimen from the lesion showed a sarcoma; the patient underwent rectosigmoidectomy. At gross inspection, the tumor measured 8 x 7 x 4 cm and was polypoid with ulcerations. Necrotic and hemorrhagic foci were scattered. Microscopically, the tumor consisted of 2 cell types: malignant tumor cells with epithelioid features and benign-appearing osteoclast-like giant cells. The tumor cells were polygonal and epithelioid in shape and had eosinophilic or clear cytoplasms, with scattered giant tumor cells. Immunohistochemical examination revealed that the tumor cells were positive for vimentin, muscle actin, alpha-smooth muscle actin, and desmin, whereas the osteoclast-like giant cells were positive for CD68, leukocyte common antigen, and lysozymes. We diagnosed this case as epithelioid leiomyosarcoma with osteoclast-like giant cells. To the best of our knowledge, this is the first case of rectal epithelioid leiomyosarcoma with osteoclast-like giant cells.     

Histological and clinical characteristics of malignant giant cell tumor of bone

Malignant giant cell tumors of bone (MGCTB) are rare, and the diagnosis can be difficult due to the occurrence of a variety of malignant tumors containing giant cells. To better understand its clinicopathological features, we have reviewed our experience with 17 cases of MGCTB. Five cases were primary malignant giant cell tumor of bone (PMGCTB), and 12 cases were giant cell tumors of bone initially diagnosed as benign but malignant in a recurrent lesion (secondary MGCTB, SMGCTB). The patients included six women and 11 men (age ranged from 17 to 52 years; mean, 30.5 years). The tumor arose in the femur (six cases), the tibia (seven cases), the humerus (three cases), and the fibula (one case). Microscopically, PMGCTB showed both conventional giant cell tumor and malignant sarcoma features. SMGCTB were initially diagnosed as conventional giant cell tumor of bone, the recurrent lesion showing malignant features. Histologically, the malignant components included osteosarcoma (11 cases), undifferentiated high-grade pleomorphic sarcoma (two cases), and fibrosarcoma (four cases). SMGCTB cases showed strong expression of p53. Follow-up information revealed that four patients died of lung metastasis, two patients are alive with lung metastases, and 11 patients are alive without tumor. MGCTB should be considered as a high-grade sarcoma. It must be distinguished from GCTB and other malignant tumors containing giant cells. p53 might play a role in the malignant transformation of GCTB.     

伴有破骨细胞样巨细胞的胰腺未分化癌1例报道及文献复习

目的：探讨伴有破骨细胞样巨细胞的胰腺未分化癌的临床病理特点.方法：观察1例伴有破骨细胞样巨细胞的胰腺未分化癌的形态学特征,并进行免疫组织化学染色.结果：伴有破骨细胞样巨细胞的胰腺未分化癌肿瘤主要有两种细胞组成,一种为单核细胞,分为组织细胞样单核细胞和梭形或多形性瘤细胞两型;另一种为多核巨细胞,分为非肿瘤性的破骨细胞样巨细胞和瘤巨细胞两型.免疫组织化学研究显示,这两种细胞Vimentin均阳性,均不表达cytokeratin（AE1/AE3）,CK5/6,CEA,CgA;其中破骨细胞样巨细胞、组织细胞样单核细胞CD45,CD68阳性,而瘤巨细胞和梭形瘤细胞阴性.结论：伴有破骨细胞样巨细胞的胰腺未分化癌是一种罕见恶性肿瘤,可能为胰腺未分化癌的一个亚型.诊断需与胰腺恶性纤维组织细胞瘤、转移的骨巨细胞瘤或黑色素瘤等鉴别.     

Malignant diffuse-type tenosynovial giant cell tumor of the buttock

Malignant diffuse-type tenosynovial giant cell tumor (D-TSGCT) is an unusual sarcoma. We report a case of malignant D-TSGCT located in the left buttock. A 58-year old woman noticed a small mass at her left buttock 3 months previously. The mass tended to enlarge rapidly, and became 6 cm in diameter. Tumor resection was performed. Grossly, the tumor showed a solid growth pattern and whitish appearance with hemorrhage and necrosis. Microscopically, the tumor was composed of a proliferation of short spindle and oval mononucleated cells with numerous osteoclast-like multinucleated giant cells, which occasionally showed coagulative necrosis. In addition, tumor cells had high mitotic activity and atypical mitoses. Immunohistochemically, the mononucleated cells were positive for CD163 and focally positive for CD68 (clone KP-1 and PG-M1), CD4, smooth muscle actin and S100 protein. Osteoclast-like multinucleated giant cells were positive for CD68 (clone KP-1 and PG-M1) and CD4. Pulmonary metastases were found 6 months after the operation. These findings indicate that this lesion is consistent with malignant D-TSGCT.     

Pathology of chronic vitamin E deficiency in fatal familial intrahepatic cholestasis (Byler disease)

Summary Three giant cell tumors of bone (2 benign and 1 malignant) were examined enzyme-histochemically, and a tissue culture study of the malignant case was performed. Multinucleated giant cells and mononuclear round cells had similar activities of ACPase and non-specific esterase with a diffuse strong reaction. ATPase and 5-nucleotidase reactions were strongly positive in the cytoplasm of multinucleated giant cells, and were seen not only in the cytoplasm but also on the cell membrane of round cells. The proliferating spindle cells in the malignant case were faintly positive for ACPase and non-specific esterase and were less positive for ATPase and 5-nucleotidase on the cell membrane. The multinucleated giant cells and mononuclear round cells resembled histiocytes in the activities of 4 hydrolytic enzymes, and the multinucleated giant cells had enzyme activities similar to those of osteoclasts from new-born rat skull.The malignant giant cell tumor and cells in its tissue culture showed ALPase activity preferentially on the cell membrane of the spindle cells, and rarely on round cells or multinucleated giant cells. ALPase was resistant to heat treatment and was found to be the type IV isoenzyme by diffusion electrophoresis. The origin of the giant cell tumor of bone and the significance of the ALPase activity are discussed.     

Giant hidradenocarcinoma: A report of malignant transformation from nodular hidradenoma

A giant hidradenocarcinoma presented by a 75-year-old female Is reported. The patient had a malignant transformation within a nodular hidradenoma involving the right postauricular area, which was treated by mass removal and a right radical neck dissection with a free-flap covering. Malignant hidradenocarcinoma is the least common adnexal tumor of uncertain origin. They are usually malignant from their inception, but some develop from a benign counterpart. To the authors' knowledge, only three cases have been reported previously. Two histologically distinct components were seen in this tumor: (i) typical nodular hidradenoma, which constituted a small part of the tumor; and (ii) carcinoma with areas of transition. The secretory cells of hidradenocarcinoma showed decapitation secretion on light and electron microscopic observations, which is evidence of apocrine differentiation. Histologically, this case was concluded as a hidradenocarcinoma arising from a long-standing nodular hidradenoma. A literature review is presented and the histological, immunohisto-chemical and ultrastructural features are described.     

Disseminated malignant fibrous histiocytoma (giant cell rich): a case report

Giant cell rich malignant fibrous histiocytoma accounts for 3 -15% of all malignant fibrous histiocytomas. Currently, the nomenclature giant cell malignant fibrous histiocytoma is reserved for undifferentiated pleomorphic sarcomas with prominent osteoclastic giant cells. It is considered to be synonymous with malignant giant cell tumor of soft parts. We report a case of disseminated giant cell malignant fibrous histiocytoma involving the scalp, cervical node, lungs, spine, abdominal wall, base of penis, gluteal cleft, paraspinal region and back. The diagnosis was established after staining for a panel of immunohistochemical markers namely cytokeratin, vimentin, S100, desmin, CD68 and smooth muscle actin. CD68 positivity in tumor cells helped in arriving at the final diagnosis. It is essential to recognize this tumor as a giant cell rich distinct entity and differentiate from other giant cell rich pleomorphic sarcomas since therapeutic and prognostic differences are being appreciated currently.     

ADRENOCORTICAL CARCINOMA IN INFANCY

A case of adrenocortical carcinoma associated with congenital heart defect in a 6-month-old Japanese girl is reported. A fist-sized tumor was incidentally noted in the right hypochondrium upon admission for cardiac surgery. No clinical endocrinopathy was evident in this case. The resected tumor was encapsulated with smooth surface and no invasion to adjacent tissues or organs was observed. Histologically, the tumor was composed of small cells with granular or clear cytoplasm, and occasional giant cells with single or multiple nuclei. By electron microscopy, the tumor cells showed various nuclear contours with distinct nucleoli and had a moderate amount of cytoplasm containing abundant rough endoplasmic reticulum and mitochondria with variable-sized electron-dense granules. Intercellular desmosome-llke junctions were observed in some tumor cells. Immunohistochemlcally, the tumor cells contained granules positive for estriol, progesterone and Cortisol. These morphological findings including electron microscopic features suggested that the tumor cells had a malignant character.     

Adrenocortical carcinoma in infancy. Report of a case with immunohistochemical and ultrastructural studies

A case of adrenocortical carcinoma associated with congenital heart defect in a 6-month-old Japanese girl is reported. A fist-sized tumor was incidentally noted in the right hypochondrium upon admission for cardiac surgery. No clinical endocrinopathy was evident in this case. The resected tumor was encapsulated with smooth surface and no invasion to adjacent tissues or organs was observed. Histologically, the tumor was composed of small cells with granular or clear cytoplasm, and occasional giant cells with single or multiple nuclei. By electron microscopy, the tumor cells showed various nuclear contours with distinct nucleoli and had a moderate amount of cytoplasm containing abundant rough endoplasmic reticulum and mitochondria with variable-sized electron-dense granules. Intercellular desmosome-like junctions were observed in some tumor cells. Immunohistochemically, the tumor cells contained granules positive for estriol, progesterone and cortisol. These morphological findings including electron microscopic features suggested that the tumor cells had a malignant character.     

Bronchogenic sarcomatoid squamous cell carcinoma with osteoclast-like giant cells

A 60-year-old man developed a widely metastatic spindle cell neoplasm with admixed osteoclast-like giant cells indistinguishable from malignant giant cell tumor of soft parts. Autopsy revealed a bronchogenic sarcomatoid squamous cell carcinoma that was the primary source of the sarcomatoid metastases. The osteoclast-like giant cells in the metastatic lesions were negative for lysozyme on immunoperoxidase staining. This finding suggested that the multinucleated giant cells were not formed as a cellular response to hemorrhage or to cellular debris induced by the tumor. Extraosseous neoplasms with osteoclast-like giant cells are rare neoplasms that may occur in a variety of organs. This case is the second reported case of a primary neoplasm in the lung that contained these osteoclast-like giant cells. These tumors may cause considerable diagnostic confusion.     

Giant cell tumor of soft tissue arising in breast

Primary giant cell tumor of soft tissue (GCT-ST) arising in breast is exceedingly rare. We report a case of a 60-year-old woman with a primary breast giant cell tumor that appeared histologically identical to giant cell tumor of bone and had a clinically malignant course. The patient presented with a cystic mass of the breast, suspected on imaging to be an organizing hematoma, possibly related to previous injury. Histopathological evaluation revealed a neoplasm composed of mononuclear cells admixed with osteoclast-like giant cells resembling giant cell tumor of bone. Immunohistochemical staining was positive for CD68, smooth muscle actin, and vimentin, but was negative for a panel of epithelial and additional muscle markers. These features were most consistent with GCT-ST, an uncommon neoplasm of low malignant potential. Despite aggressive surgical treatment achieving clear surgical margins, the patient expired with pulmonary metastases within a year of her initial presentation. This case demonstrates the difficulty of predicting clinical behavior of GCT-ST of breast on the basis of histological features and depth of tumor alone. To our knowledge, this is the first case report of a GCT-ST arising in the breast associated with a fatal outcome. The distinction of this entity from other more common primary breast tumors with giant cell morphology is also emphasized.     

Combined hepatocellular carcinoma and osteoclast-like giant cell tumor of the liver: Possible clue to histogenesis

Hepatic giant cell tumor is extremely rare, and only five cases have been reported of overt hepatocellular carcinoma, thus its histogenesis is controversial. Herein is reported a case of simultaneous hepatocellular carcinoma and osteoclast-like giant cell tumor in a single tumor. A liver tumor was found in a 74-year-old woman. Histologically the tumor consisted of two distinct components: mononuclear and multinuclear giant cells with osteoclastic giant cells, and a conventional hepatocellular carcinoma. The boundary between the two components showed transitional features. Immunohistochemistry showed that the osteoclast-like giant cells were CD68 and vimentin positive, but cytokeratin and AFP negative, while spindle-shaped cells were positive only for vimentin. In a portion of the hepatocellular carcinoma the cells were cytokeratin-8 and AFP positive. Ki-67 positivity was 10% for the hepatocellular carcinoma, 60% for the spindle-shaped cells, and 0% for the giant cells. It is possible that the tumor might have had a hepatocellular carcinoma origin, given the more highly proliferative sarcomatous changes and reactive osteoclast-like cells. This case provides a clue to the histogenesis of hepatic giant cell tumors.     

Malignant meningioma with cartilage and giant cells.

An autopsy case of recurrent and malignant meningioma is reported. This case was originally typical benign transitional meningioma of the falx, however, the histology of the tumor changed to show malignant features during successive recurrences. At autopsy, the tumor revealed findings consistent with malignant meningioma. One of the most interesting features was the presence of cartilage and giant cells in some parts. Immunohistochemistry showed positive immunoreactivity for S-100 protein in some cartilage and giant cells and for cytokeratin in some giant cells. Multidifferential potential of the meningioma cells was suggested in this case.     

Malignant Meningioma with Cartilage and Giant Cells

An autopsy case of recurrent and malignant meningioma is reported. This case was originally typical benign transitional meningioma of the falx, however, the histology of the tumor changed to show malignant features during successive recurrences. At autopsy, the tumor revealed findings consistent with malignant meningioma. One of the most interesting features was the presence of cartilage and giant cells in some parts. Immunohistochemistry showed positive immunoreactivity for S-100 protein in some cartilage and giant cells and for cytokeratin in some giant cells. Multi-differential potential of the meningioma cells was suggested in this case.     

Malignant giant cell tumor of tendon sheath. Report of a case

In a patient with pigmented villonodular synovitis of the right knee joint, there occurred a malignant giant cell tumor of tendon sheath. There was clinical evidence of metastasis after the second local recurrence and the recurrent tumors were studied enzyme cytochemically and electron microscopically. Ultrastructurally, the malignant tumor consisted of three principal cell types; histiocyte-like cells, fibroblast-like cells, and intermediate cells, with unique attendance of myofibroblasts. This may be the first report of the presence of myofibroblasts in malignant giant cell tumor of tendon sheath. Enzyme cytochemistry revealed various functional properties of histiocytes.