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Alberto G Ayala

Publications and source records attributed to Alberto G Ayala.

At least 19 recordsLinked to original sources
PubMed2006

Characterization of the myxoid variant of hibernoma.

Hibernomas are rare benign neoplasms composed of brown adipose tissue. Although they were first mentioned in the literature almost a century ago, relatively few reports have documented their range of pathological features. Multivacuolated fat cells with small, central nuclei are typical features of hibernomas. Based on the nature of the stroma and the appearance of the multivacuolated cells, 4 categories of hibernomas are described: typical, lipoma-like, myxoid, and spindle cell. The myxoid variant is very rare. In this report, we present and characterize the spectrum of morphological features of the myxoid variant of hibernoma by describing the case of a 45-year-old white man who was referred to our hospital for treatment of an ovoid mass in the posterior segment of the right deltoid region. Histopathologic examination of the resected specimen revealed a multilobulated, tan-yellow, variegated tumor with a rubbery consistency that was separated by thick fibrous septa with interspersed collections of foamy histiocytes. The mass was initially diagnosed as schwannoma with degenerative changes, but workup revealed a myxoid variant of hibernoma. This report highlights recent advances that may help confirm the diagnosis and explain the differential diagnosis of this rare tumor. This is one of only a few cases of a myxoid variant of hibernoma reported in the literature.

Biomarkers, Tumor↗
PubMed2006

Thyroid follicular carcinoma-like tumor of kidney: a case report with morphologic, immunohistochemical, and genetic analysis.

We present an unusual renal tumor, which has not been classified under a known subtype of renal cell carcinoma (RCC) and characteristically shows similar histology to thyroid follicular carcinoma. The patient was a 32-year-old asymptomatic woman who was found to have a kidney mass during her annual physical examination. She had no lesions in the thyroid during physical and ultrasound examinations, and there was no abnormal thyroid function test. Neither mediastinal nor ovarian abnormalities were observed. The resected kidney showed a well-defined nodular tumor measuring 11.8x8.0x8.0 cm. The mass was protruding into the pelvic cavity with areas of yellowish geographic necrosis. Histologically, the tumor showed follicular architectures with inspissated colloid-like material in their lumina. No conventional (clear cell) RCC or any other known subtypes of RCC component was observed. Immunohistochemically, the tumor cells showed intensive staining for cytokeratin (CK) cocktail AE1/AE3 and CD10 and were not reactive to thyroid transcription factor-1 and thyroglobulin. The staining of CK35betaH11 and vimentin revealed focal cytoplasmic reaction. The tumor cells were completely negative for CK7, CK19, CK20, CK34betaE12, carcinoembryonic antigen, epithelial membrane antigen, and CD15. Chromosomal gains of 7q36, 8q24, 12, 16, 17p11-q11, 17q24, 19q, 20q13, 21q22.3, and Xp and losses of 1p36, 3, and 9q21-33 were detected by comparative genomic hybridization. These findings are dissimilar to previously classified renal neoplasm. Only a report that included three cases of primary thyroid-like renal tumor has been described in the abstract form. However, there is no fully documented case on this unusual form of RCC, which morphologically resembles that of thyroid follicular carcinoma. Herein, we present a new case of thyroid follicular carcinoma-like tumor of the kidney with a chromosomal study and review of the literature.

Adenocarcinoma, Follicular↗
PubMed2006

Juxtaglomerular cell tumor of kidney with CD34 and CD117 immunoreactivity: report of 5 cases.

CONTEXT: Juxtaglomerular cell tumor is a rare renal neoplasm. Renin immunohistochemistry and electron microscopic documentation of rhomboid crystals are the primary methods of diagnosing this benign tumor. OBJECTIVES: In this retrospective study, we evaluated the morphologic, immunohistochemical, and ultrastructural features of 5 cases of juxtaglomerular cell tumor to determine the effectiveness of CD34 and CD117 immunohistochemistry for the diagnosis of this tumor. DESIGN: We reviewed 5 cases with clinical, histologic, immunohistochemical, and ultrastructural aspects. RESULTS: Three women and 2 men with a mean age of 37.8 years (range, 16-60 years) were included in this study. All patients presented with severe hypertension. All tumors were well circumscribed and ranged from 1.5 cm to 8.5 cm (mean, 4.4 cm). On light microscopic examination, we found solid sheets and nests of tumor cells with oval-to-round nuclei and eosinophilic cytoplasm. Low-power microscopic examination disclosed a hemangiopericytic vascular pattern. Immunohistochemistry results were as follows: vimentin (positive), renin (weakly positive), smooth muscle actin (focal immunoreactivity), and cytokeratin (negative). All 5 tumors were immunoreactive for CD34 and CD117. Electron microscopy revealed rhomboid crystals in the cytoplasm. Postoperatively, 4 patients were normotensive and 1 patient experienced persistent mild hypertension. CONCLUSIONS: Our findings indicate that immunohistochemistry for CD34 and CD117 are effective at diagnosing juxtaglomerular cell tumor. Juxtaglomerular cell tumor should be considered in the diagnosis of any renal tumors with epithelioid cells and negative initial cytokeratin immunohistochemistry.

Adenocarcinoma↗
PubMed2006

Mucinous tubular and spindle cell carcinoma of the kidney with neuroendocrine differentiation: report of two cases.

We encountered 2 cases of mucinous tubular and spindle cell carcinoma (MTSCC) during a short time. In a 61-year-old man who had macroscopic hematuria for 1 month, the 14.5 x 14.0 x 12.0-cm resected tumor involved the right middle aspect of the renal parenchyma and compressed the renal pelvis. In an asymptomatic 47-year-old man, a renal tumor was found during an annual physical examination. The 3.5 x 3.0 x 2.0-cm tumor was located at the upper pole of the right kidney. The histologic findings in both cases were similar Tumors consisted of tightly packed, small, elongated tubules separated by pale mucinous stroma. The tumor cells were cuboidal to spindled with eosinophilic cytoplasm and low nuclear grade. Mitoses were few or nonexistent and without abnormal figures. Both tumors were immunoreactive for cytokeratin (CK) cocktail (AE1/AE3), high-molecular-weight CK (34betaE12), low-molecular-weight CK (35betaH11), CK7, epithelial membrane antigen, E-cadherin, and vimentin. The tumor cells also were reactive for neuron-specific enolase, chromogranin, and synaptophysin. The ultrastructure of the tumor cells contained abundant mitochondria, junctional complex, and dense-core neurosecretory granules. We present 2 additional cases of MTSCC showing typical morphologic features with neuroendocrine differentiation.

Adenocarcinoma, Mucinous↗
PubMed2005

Adenomatoid tumor of the adrenal gland: a clinicopathologic study of 3 cases.

Adenomatoid tumors are relatively uncommon benign neoplasms of mesothelial origin, usually occurring in the male and female genital tracts. Rare extragenital adenomatoid tumors have been identified in the adrenal glands, heart, mesentery, pleura, and lymph nodes. In the adrenal gland, adenomatoid tumors may pose a diagnostic challenge. The differential diagnosis includes adrenocortical carcinoma and metastatic carcinoma, especially signet ring cell carcinoma. Because of its glandular pattern, an adenomatoid tumor may be confused with an adenocarcinoma. We present 3 cases of adrenal adenomatoid tumors, including one with a concurrent large hemorrhagic vascular adrenal cyst. The adenomatoid tumors were unilateral, appeared solid and white, and varied from 1.7 to 4.2 cm in diameter. They occurred in 3 male patients aged 33, 33, and 46 years. One patient presented with abdominal pain due to the presence of a concurrent large adrenal cyst. The tumor was an incidental radiological finding in another case and was discovered during the course of a workup for hypertension in the third case. The light microscopic appearances were consistent with those of typical adenomatoid tumors. Immunohistochemical stains for calretinin and cytokeratin 5/6 were positive, confirming the tumors' mesothelial origin. Ultrastructural studies performed in 2 cases revealed microvilli and desmosomes. Follow-up showed no evidence of recurrence or metastasis. In our experience, the key to the diagnosis of this rare benign tumor is to consider adenomatoid tumor in the differential diagnosis of any glandular tumor occurring in the adrenal gland.

Adenocarcinoma↗
PubMed2004

Lymphoepithelioma-like carcinoma of the breast: report of a case mimicking lymphoma.

Only 10 cases of lymphoepithelioma-like carcinoma of the breast have been reported in the literature. This report adds one more case to the published literature. A 62-year-old woman presented with a mass in her left breast on physical examination. The mammographic images showed a 3.0 cm, poorly defined mass in the upper outer quadrant. A biopsy was recommended. The gross specimen consisted of a 5 cm portion of breast parenchyma with no discrete tumor. On microscopic examination, the tumor was composed of sheets of epithelioid cells arranged as single cells or in cords partially obscured by a dense lymphocytic infiltrate. The epitheliod cells extensively expressed cytokeratin stain, but did not express E-cadherin. The lymphoid cells expressed L26 stain in the germinal centers, and CD3 stain in the T lymphocytes surrounding the germinal centers and in between tumor cells. In situ hybridization showed no evidence of Epstein-Barr virus infection in the tumor cells. An overall review of 11 cases shows that the disease is usually seen in older patients. In situ and invasive lobular component was reported in 36% of the cases. Eight of 11 were negative for E-cadherin, 36% were estrogen receptor-positive, 18% were progesterone receptor-positive, and all of them were HER2/neu negative. None of the reported cases have been associated with Epstein-Barr virus infection. Only two of the cases showed lymph node metastasis, and long-term follow-up in one of them showed good prognosis. In summary, lymphoepithelioma-like carcinoma of the breast is a tumor with a good prognosis that should be considered as a possible diagnosis in breast tumors with an intense lymphocytic infiltrate.

Biomarkers, Tumor↗
PubMed2004

Carcinoma of lung with rhabdoid features.

Lung tumors with rhabdoid features, included as variants of large cell carcinoma in the 1999 World Health Organization classification of lung tumors, are rare and have an aggressive clinical course. We report 11 patients with primary lung tumors with rhabdoid features and review the literature on this uncommon tumor. We examined samples from 7 primary (6 resections, 1 biopsy) and 4 metastatic tumor samples. All specimens were stained with immunohistochemical stains for pancytokeratin (CK), cytokeratin 7 (CK7), cytokeratin (CK20), thyroid transcription factor-1 (TTF-1), and vimentin. The patients were 7 men and 4 women whose ages ranged from 35 to 70 years. Nine patients presented with respiratory symptoms, and 9 patients had a history of heavy smoking. One patient had TNM stage I tumor, 3 had stage III tumors, and 6 had stage IV tumors at presentation; tumor stage could not be determined in 1 patient. Histological examination of these tumors showed typical rhabdoid cells: large cells with abundant cytoplasm, a large eccentric nucleus with a central macronucleolus, and a rounded eosinophilic cytoplasmic inclusion that sometimes caused nuclear indentation. These cells constituted 10% to 90% of the tumor. The "parent" neoplasm was sarcomatoid carcinoma and adenocarcinoma in 4 cases each and was large cell undifferentiated carcinoma in 3 cases. Cytoplasmic staining in the rhabdoid cells was seen in 9 of 11 cases for CK, in 4 of 10 cases for CK7, and in all 11 cases for vimentin. Nuclear staining for TTF-1 in the rhabdoid cells was absent in all 11 cases, and cytoplasmic staining for CK20 was negative in the rhabdoid cells in all 10 cases studied. Of the 9 patients with available follow-up information, 8 died of disease, and 1 is alive with no evidence of disease 20 months after the initial diagnosis. We conclude that rhabdoid features can occur in a variety of lung tumors, including sarcomatoid carcinoma. Recognizing these lesions is important because of their possibly aggressive clinical course.

Adenocarcinoma↗
PubMed2004

Risk-adapted treatment for patients with clinical stage I nonseminomatous germ cell tumor of the testis.

OBJECTIVES: To evaluate whether two courses of chemotherapy after orchiectomy in patients with clinical Stage I nonseminomatous germ cell testicular tumor at high risk of relapse will spare patients additional chemotherapy or surgery. METHODS: High-risk patients had one or more of the following: preorchiectomy alpha-fetoprotein level of 80 ng/dL or greater, 80% embryonal cell carcinoma or greater, or vessel invasion in the primary tumor. Low-risk patients had none of these factors or had 50% teratoma or more without vessel invasion. High-risk patients were offered two 21-day courses of outpatient chemotherapy consisting of carboplatin, etoposide, and bleomycin. Low-risk patients and high-risk patients not receiving chemotherapy were observed. RESULTS: Of 99 patients, we classified 76 as high risk and 23 as low risk of relapse. All but eight of the high-risk patients received chemotherapy. No patient who underwent chemotherapy developed relapse, although 1 patient with normal biomarkers and a late-appearing mass underwent retroperitoneal lymphadenectomy for mature teratoma. Two of the 23 low-risk patients had disease relapse; both successfully underwent chemotherapy. The nonhematologic toxicity was mild in patients receiving chemotherapy, and no patient required hospitalization. The median follow-up was 38 months (range 9 to 69). CONCLUSIONS: Two courses of postorchiectomy adjuvant chemotherapy were safe and well tolerated and markedly decreased the relapse rate in high-risk patients with clinical Stage I nonseminomatous germ cell testicular tumor without additional surgery or more protracted chemotherapy. This approach may avoid potential problems with compliance and diminish the cost of scrupulous follow-up. Our results support that surveillance for carefully selected patients at a low risk of relapse is appropriate.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2004

Can the number of cores with high-grade prostate intraepithelial neoplasia predict cancer in men who undergo repeat biopsy?

OBJECTIVES: To evaluate whether the presence of, or the number of cores containing, high-grade prostatic intraepithelial neoplasia (PIN) found in men who underwent initial extended multisite biopsy could predict which men would have prostate cancer on subsequent repeat biopsies. METHODS: Between June 1997 and January 2003, 1086 men underwent initial prostate biopsy for early detection of prostate cancer using an extended multisite biopsy scheme. Of these, 175 men without cancer underwent at least one repeat biopsy (range one to three; median interval between biopsies, 3 months). Among these 175 patients, 47 had high-grade PIN on initial biopsy. RESULTS: The initial extended biopsy identified cancer in 33.8% (367 of 1086) and high-grade PIN in 20.8% (226 of 1086). The incidence of high-grade PIN only in patients found to have cancer on initial biopsy was 29.7% (109 of 367). The presence of high-grade PIN was associated with concurrent prostate cancer at the initial biopsy (P <0.0001). Overall, repeat biopsy identified cancer in 18.3% of the 175 men. Of the 47 men with high-grade PIN, 5 (10.6%) were found to have cancer on repeat biopsy. The number of biopsy specimens positive for high-grade PIN on initial biopsy was not associated with the likelihood of prostate cancer on repeat biopsy. Multivariate logistic regression analysis showed that neither the presence of high-grade PIN nor the number of cores containing high-grade PIN on initial biopsy were predictors for prostate cancer on repeat biopsy. CONCLUSIONS: The number of cores positive for high-grade PIN was not predictive for cancer on repeat biopsy.

Adenocarcinoma↗
PubMed2004

Breast carcinoma with choriocarcinomatous features.

Although breast carcinomas have been shown to produce various ectopic substances, including human chorionic gonadotropin, it is rare to identify morphologic differentiation compatible with the hormone produced by a tumor. Presently, only eight cases of breast carcinoma with focal choriocarcinomatous differentiation have been reported in the literature. This article describes the pathologic findings, immunohistochemical profile, and clinical course in two additional cases of this unusual variant of breast carcinoma. In the first case, the tumor had morphologic features suggestive of medullary carcinoma, and the patient is doing well 12 months after presentation. In the second case, the tumor was locally advanced at presentation with histologic features consistent with metaplastic carcinoma having squamous, sarcomatoid, and choriocarcinomatous elements. The patient presented with extensive multifocal metastases 6 months after the initial presentation and is not responding well to standard or experimental treatment regimen. Immunostaining for the beta subunit of human chorionic gonadotropin was localized mostly, but not entirely, to multinucleated syncytiotrophoblast-like giant cells within both tumors.

Adult↗
PubMed2004

Synovial sarcoma of the kidney with rhabdoid features: report of three cases.

We report 3 cases of synovial sarcoma with rhabdoid features, initially diagnosed as adult rhabdoid tumors. Two women (case nos. 1 and 2, 35 years and 27 years of age, respectively) and one man (case no. 3, 26 years of age) presented to their physicians with right flank pain. On physical examination, a poorly defined, firm, palpable mass was found in the upper right quadrant of the abdomen in all cases. Sonography and computed tomography revealed solid, cystic masses in the right kidneys that ranged in size from 8.5 to 20.0 cm. Right radical nephrectomies were performed in all patients. One patient died of disease, and the other two patients were alive and disease-free after chemotherapy and radiotherapy. Microscopic examination revealed that the tumors were composed mostly of rhabdoid cells with eccentrically located nuclei, prominent nucleoli, and eosinophilic cytoplasm. We also found areas of fasciculated spindle cells, sharply separated from or irregularly admixed with areas of rhabdoid cells. There was tumor necrosis, but no epithelial areas were seen. Hemangiopericytic vasculature was at least focally observed in all cases. The tumor cells were positive for CD99 and bcl-2 in all cases and for CD56 in two cases and negative for CD34 and smooth muscle actin in all cases. The cells in case no. 1 were focally positive for cytokeratin. To verify the possibility of synovial sarcoma with rhabdoid features, reverse transcriptase polymerase chain reaction using RNA extracted from frozen tissue in case no. 1 and formalin-fixed, paraffin-embedded tissue in case nos. 2 and 3 was performed. SYT-SSX2 transcripts were detected in all 3 cases. These cases indicate that synovial sarcoma of the kidney should be considered in the differential diagnosis of mesenchymal kidney tumors with prominent rhabdoid features. A subset of adult rhabdoid tumors may be a rhabdoid variant of synovial sarcoma, and molecular studies to detect SYT-SSX fusion transcripts are recommended for an accurate diagnosis.

Adult↗
PubMed2004

Ewing sarcoma/peripheral primitive neuroectodermal tumor: adult abdominal tumors with an Ewing sarcoma gene rearrangement demonstrated by fluorescence in situ hybridization in paraffin sections.

The differential diagnosis of small round cell tumors is exhaustive and requires ancillary studies. Relatively recently, fluorescence in situ hybridization (FISH) using probes for specific gene rearrangements has gained wide acceptance. This technique is particularly useful in the differential diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) and desmoplastic small round-cell tumor (DSRCT). In ES/PNET, the EWS gene is juxtaposed to the FLI-1 gene in 85% of cases and to the ERG gene in another 7% of cases; the EWS gene is juxtaposed to the WTI gene in DSRCT. Documentation of the EWS gene rearrangements in EWS/PNET has previously been demonstrated in frozen tissue. We report 2 unusual cases of EWS/PNET diagnosed in abdominal tumors in adults. Although the immunohistochemical results supported a diagnosis of ES/PNET, 1 case morphologically resembled DSRCT. The diagnosis in these 2 cases was confirmed by the FISH demonstration of EWS/FLI-1 gene fusion in paraffin-embedded tissue. Thus, the usefulness of FISH demonstration of an EWS gene rearrangement with these specific probes in such unusual cases is supported and is demonstrated in paraffin-embedded tissue.

Abdominal Neoplasms↗
PubMed2003

Presurgical chemotherapy compared with immediate surgery and adjuvant chemotherapy for nonmetastatic osteosarcoma: Pediatric Oncology Group Study POG-8651.

PURPOSE: Successful therapeutic interventions to prevent disease progression in patients with nonmetastatic osteosarcoma have included surgery with adjuvant chemotherapy. Presurgical chemotherapy has been advocated for these patients because of putative improvement in event-free survival (EFS). The advantages of presurgical chemotherapy include early administration of systemic chemotherapy, shrinkage of primary tumor, and pathologic identification of risk groups. The theoretic disadvantage is that it exposes a large tumor burden to marginally effective chemotherapy. The contribution of chemotherapy and surgery timing has not been tested rigorously. PATIENTS AND METHODS: Between 1986 and 1993, we conducted a prospective trial in patients with nonmetastatic osteosarcoma who were assigned randomly to immediate surgery or presurgical chemotherapy. Except for the timing of surgery (week 0 or 10), patients received 44 weeks of identical combination chemotherapy that included high-dose methotrexate with leucovorin rescue, doxorubicin, cisplatin, bleomycin, cyclophosphamide, and dactinomycin. RESULTS: One hundred six patients were enrolled onto this study. Six were excluded from analysis. Of the remaining 100 patients, 45 were randomly assigned to immediate chemotherapy, and 55 were randomly assigned to immediate surgery. Sixty-seven patients remain disease-free. At 5 years, the projected EFS +/- SE is 65% +/- 6% (69% +/- 8% for immediate surgery and 61% +/- 8% for presurgical chemotherapy; P =.8). The treatment arms had similar incidence of limb salvage (55% for immediate surgery and 50% for presurgical chemotherapy). CONCLUSION: Chemotherapy was effective in both treatment groups. There was no advantage in EFS for patients given presurgical chemotherapy.

Adolescent↗
PubMed2003

Triple synchronous neoplasms in one kidney: report of a case and review of the literature.

We report the case of a patient with three synchronous but histologically different primary renal tumors that were all in the same kidney. Two tumors were different subtypes of renal cell carcinoma (RCC), and the third was a variant form of angiomyolipoma. The patient was a 62-year-old man who was receiving antihypertensive drugs and came to our hospital for a regular check-up. Ultrasonography performed during the visit revealed a left renal mass, but the patient had no related symptoms. Subsequent computed tomography revealed two round, high-density masses, one in the mid-portion and the other in the lower pole of the left kidney, and multiple cysts in the right kidney and the liver. The mass in the mid-portion measured 3.3 x 3.0 x 2.8 cm, and the mass in the lower pole measured 1.7 x 1.1 x 0.9 cm. A left radical nephrectomy was performed. On gross examination, an additional ovoid nodule (0.6 cm in the greatest dimension) was found in the lower pole. Microscopically, the largest tumor consisted of a broad alveolar arrangement of large round cells with abundant eosinophilic or clear cytoplasm, distinct cell borders, and perinuclear halos, features consistent with chromophobe RCC. The smallest tumor was a conventional (clear-cell) RCC. The third tumor was composed solely of atypical epithelioid cells with prominent nucleoli and yellowish-brown to black pigments. The tumor cells were positive for melanin (Fontana-Masson stain), the melanoma marker HMB45, vimentin, smooth-muscle actin, and the macrophage marker CD68 and were negative for cytokeratin. This tumor was considered a pigmented epithelioid type of angiomyolipoma. The histologic, histochemical, and immunohistochemical features in this case confirmed the presence of three synchronous primary tumors, a chromophobe and a clear-cell type RCC and a pigmented epithelioid angiomyolipoma, all of which were in the same kidney. This case is the first of its type reported in the literature.

Angiomyolipoma↗
PubMed2003

Vascular tumors of bone: A study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid hemangioendothelioma, and high-grade angiosarcoma.

Cases filed as vascular tumor of bone other than ordinary hemangioma were reviewed. They were included in the study if there was adequate histologic material and clinical information, clear evidence of bone origin, and at least 5 years follow-up. The study group comprised 17 cases, of which 13 were categorized as hemangioendothelioma of bone, 1 as epithelioid hemangioendothelioma, and 3 as high-grade angiosarcoma. Hemangioendothelioma of bone had growth patterns varying from vasoformative to solid, but well-formed vessels were present in at least some area in all cases. The cells generally had a rounded, epithelioid character, regular nuclei, and relatively few mitotic figures; occasional features included spindle cells and scattered enlarged, hyperchromatic or pleomorphic nuclei. Lymphoplasmacytic and eosinophilic inflammatory infiltrate ranged from prominent to slight or absent, and myxoid or hyaline stroma was never more than focal. Epithelioid hemangioma could not be separated from hemangioendothelioma of bone. The single epithelioid hemangioendothelioma for the most part had cords of relatively uniform epithelioid cells in a prominent myxoid stroma but focally demonstrated an angiosarcoma-like appearance, with irregular vascular spaces and marked nuclear pleomorphism. The high-grade angiosarcomas exhibited predominantly irregular vasoformation combined with solid areas, diffuse nuclear hyperchromatism and pleomorphism, and, in 2 cases, numerous mitotic figures (the third case had only a small biopsy and a postradiation amputation specimen). Of the hemangioendotheliomas of bone, 7 were unicentric and 6 were regionally multicentric either concurrently or sequentially. Three patients had intraosseous local recurrence, 2 had discontinuous regional skin or soft tissue involvement (including the popliteal artery in 1), and 1 had a solitary lung metastasis, but none died of tumor. The patient with epithelioid hemangioendothelioma had multicentric tumors in widely separated bones and died with liver and lung metastases. Two of the high-grade angiosarcomas were unicentric, and the third was regionally multicentric, with a popliteal artery-soft tissue component as well. All 3 of these patients died with metastases in various sites.

Adolescent↗
PubMed2003

Pulmonary epithelioid hemangioendothelioma with prominent signet ring cell features mimicking metastatic adenocarcinoma.

We report the case of a 65-year-old woman who presented with persistent dry cough and was initially treated for pneumonia without much improvement. Computed tomography of the chest showed a 2.5 cm poorly marginated right infrahilar opacity and multiple ill-defined ground glass opacities scattered throughout the lungs. Computed tomography-guided fine needle aspiration of the right infrahilarmass showed malignant epithelioid cells, many of which were strikingly plasmacytoid with a vacuolated cytoplasm suggesting signet ring cells. A diagnosis of adenocarcinoma with signet ring cell features was made and, in view of the clinical history of multiple masses in the lung, metastatic adenocarcinoma from sites such as gastrointestinal or pancreaticobiliary tract was favored. The patient underwent exploratory thoracotomy during which she was found to have multiple, small pulmonary nodules, one of which was sampled for intraoperative examination. Frozen section of the nodule was interpreted as adenocarcinoma with signet ring cell features, probably metastatic in origin. Histopathologic examination of the pulmonary nodules demonstrated fairly well-delineated lesions with an acellular central zone of hyalinized matrix surrounded by a more cellular zone of moderately pleomorphic epithelioid cells, some of which had a peripherally displaced nucleus and vacuolated cytoplasm resembling signet ring cells. The epithelioid cells were positive for vascular markers CD31 and CD34 and, therefore, a diagnosis of epithelioid hemangioendothelioma was confirmed. This case clearly indicates the importance of recognizing the cytomorphologic and histologic spectrum of this entity to avoid misdiagnosing it as either primary or metastatic adenocarcinoma.

Aged↗
PubMed2003

Chromophobe renal cell carcinoma with sarcomatoid transformation.

We present a rare case of a chromophobe renal cell carcinoma that progressed to a high-grade spindle cell sarcoma. The tumor affected a 50-year-old man who had presented with right upper quadrant discomfort and hematuria and subsequently underwent a right radical nephrectomy. Microscopically, the tumor was composed of two distinct components, a chromophobe renal cell carcinoma and a sarcomatoid component. The sarcomatoid component had exhibited aggressive behavior by spreading to a regional lymph node. This case report shows that chromophobe carcinoma can develop a sarcomatoid transformation with a high propensity for invasive growth and metastasis.

Carcinoma, Renal Cell↗