Histiocytic Markers Research Articles

A Rare Case of Erdheim-Chester Disease

Erdheim-Chester disease (ECD) is a rare disease characterized by the abnormal proliferation of tissue macrophages. Usually, onset is in middle age. Most patients have osseous involvement with generalized sclerosis of the long bones. Heart, lung, CNS, and skin are also frequently involved. ECD cells express the histiocyte marker CD68, but unlike Langerhans cell histiocytosis, …

Abstract 3863: Transgenic model for early prostate metastasis to the lymph nodes.

Abstract Background: The emergence of recurrent, metastatic prostate cancer following the failure of androgen-deprivation therapy represents the lethal phenotype of this disease. However, little is known regarding the genes and pathways that regulate this metastatic process, and moreover, it is unclear whether metastasis is an early or late event. The SSeCKS/Gravin/AKAP12 (“SSeCKS”) gene, previously shown to suppress prostate cancer metastasis upon re-expression (1,2), as well as the tumor suppressor gene, Rb, are either transcriptionally downregulated or deleted in human prostate cancers (3,4). Mouse models deficient in either SSeCKS or Rb exhibit prostatic hyperplasia (5,6). Moreover, SSeCKS-null fibroblasts as well as SSeCKS-null prostates display Rb-dependent premature senescence markers (7). Methods: In order to determine whether the combined loss of AKAP12 and Rb in the prostate synergizes to induce oncogenic progression, Akap12-/- mice were crossed with Pb4-Cre;RbloxP/loxP mice to generate Akap12-/-;RbPE-/− progeny, with the Probasin-Cre passed only through males. Results: The combined loss of SSeCKS and Rb results in prostatic intraepithelial neoplasia (PIN) starting at 6 months of age that fails to progress to malignancy (adenocarcinoma) after 18 months. The PIN lesions were marked by increased Ki-67 proliferation of cytokeratin 8 (CK8), p63-negative luminal cells as well as p63-positive basal cells. Interestingly, these lesions also had increased numbers of androgen-receptor (AR)-positive, p63-positive, CK5-negative cells. There was evidence of reactive stroma including mural hyperplasia and inflammatory cell infiltration. Strikingly, 83% of mice with PIN lesions exhibited metastases to draining lymph nodes (LN), marked by well-differentiated tumors cells expressing markers of basal (p63, CK14) and luminal (CK8 and AR) epithelial cells, although none expressed the basal marker, CK5. PCR-based tests for the deleted floxed Rb allele from laser capture microdissected LN lesions confirmed the prostatic origin of these metastatic cells. The LN lesions showed very limited inflammation, based on very few cells staining with the histiocyte marker, CD68. Conclusions: Taken together, these data suggest that in the context of Rb loss, SSeCKS suppresses the oncogenic proliferation and early metastatic spread of transitional, basal-luminal prostate tumor cells. Citation Format: Hyun-Kyung Ko, Shin Akakura, Jennifer Peresie, David W. Goodrich, Barbara A. Foster, Irwin H. Gelman. Transgenic model for early prostate metastasis to the lymph nodes. [abstract]. In: Proceedings of the 104th Annual Meeting of the American Association for Cancer Research; 2013 Apr 6-10; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2013;73(8 Suppl):Abstract nr 3863. doi:10.1158/1538-7445.AM2013-3863

Littoral Cell Angiosarcoma in a Dog

This report describes the microscopical and immunohistochemical characteristics of littoral cell angiosarcoma in a 12-year-old, neutered female, beagle dog. The dog succumbed to metastatic disease 3 months after diagnosis of a mid-splenic mass. The tumour was characterized by two histological patterns: anastomosing microvascular channels and microvascular papillary fronds. The neoplastic cells expressed both endothelial and histiocytic markers and were erythrophagocytic. Immunohistochemical findings consistent with malignancy were CD34 expression and high Ki67 nuclear immunoreactivity.

Atypical fibroxanthoma—a diagnosis of exclusion!

Fibrohistiocytic tumors of the skin comprise a large range of lesions. One such tumor is the atypical fibroxanthoma (AFX), which is widely considered as a "pseudomalignant" tumor. It is derived from fibroblasts and expresses a variety of histiocytic markers. We present a case of AFX, localized in the right temporal region of the scalp, successfully treated with surgical excision. Immunohistochemical staining helps differentiate this tumor from others in the clinical differential diagnosis, including malignant melanoma, squamous cell carcinoma, and other nonmelanocytic spindle cell tumors such as leiomyosarcoma, rhabdomyosarcoma, angiosarcoma, liposarcoma, and dermatofibrosarcoma protuberans. Historically, AFX was believed to be a superficial variant of malignant fibrous histiocytoma (MFH). However, MFH is now considered a more generalized term for a sarcomatous neoplasm of the subcutaneous tissue. The histopathology of MFH shares features with some malignant mesenchymal neoplasms such as liposarcoma, leiomyosarcoma, rhabdomyosarcoma, and angiosarcoma, but can be differentiated using immunohistochemistry and/or electron microscopy. More recently, the examples of MFH that do not exhibit a more specific line of differentiation have been reclassified as undifferentiated pleomorphic sarcoma (UPS). Many authors currently cannot draw a distinction between AFX and UPS. The clinical and histopathological differences between AFX and UPS are often difficult to delineate. It is probable that they represent two poles of the same disease. Surgical excision in the patient we describe resulted in excellent aesthetic results with lack of recurrence in the 7-month postoperative period.

Solitary juvenile xanthogranuloma in cervical spine: case report and review of literature

Solitary juvenile xanthogranuloma (JXG) in the spinal column is extremely rare. Here, we report and characterize the case of xanthogranuloma of the upper cervical spine. A 18-year-old male presented with neck pain for 3 months, along with progressive quadriparesis and sensory loss of 2 months duration with urinary retention. Motor examination revealed spastic quadriparesis with power of 2/5 in all the 4 limbs. Magnetic Resonance Imaging (MRI) spine with contrast showed a dorsally placed intradural extramedullary lesion at the level of C2-C4 vertebral body. The lesion, measuring 2.9x1.7x1.4 cm, was isointense on T1WI, hypointense on T2WI, and enhanced homogenously on contrast. He underwent an emergency C2-C4 laminectomy and complete excision of the lesion. At 3-month follow-up, he was asymptomatic except for mild neck pain. MRI scan of the cervical spine done at follow-up, revealed complete excision of tumor without any residual lesion. Histopathological examination of the mass revealed a polymorphous population of sheets of bloated pale foamy histiocytes (xanthoma cells), numerous admixed mature lymphocytes and several Touton giant cells. The cells were positive for CD68, a histiocytic marker, and negative for CD1a (excludes LCH) and S-100 (excludes RDD).

Isolated Juvenile Xanthogranuloma in the Larynx of a Three-Year-Old Child

Juvenile xanthogranuloma (JXG) is a benign manifestation of non-Langerhans cell histiocytosis characterized by yellowish cutaneous nodules. Its occurrence in the larynx is very rare, but laryngeal JXG may cause severe respiratory distress. We report a patient with isolated laryngeal JXG treated by laryngomicrosurgery, and this is the first report of JXG extending to vocal fold. A 3-year-old girl presented with hoarseness and inspiration stridor. A bulky tumor was found in right glottic to subglottic region. Subtotal resection of the tumor was carried out by laryngomicrosurgery, and airway distress was diminished after the operation. In pathological examination, the resected specimen showed proliferation of histiocytic cells and spindle cells with Touton giant cells that are characterized by polynuclei or wreath nuclei and are known to appear in JXG but not in LCH. Immunohistochemistry of histiocytic cell markers demonstrated positivity for CD68, lysozyme, alpha1-anti-chymotrypsin, factor XIIIa and vimentin, and negativity for CD1a and S-100, leading to diagnosis of JXG, but not LCH. The patient was thus expected with benign prognosis, and additional resection of the tumor including vocal fold was not indicated in the initial treatment. Six weeks later, the JXG recurred and a second procedure using CO₂ laser was needed. The tumor did not re-grow thereafter, and there was no residual voice handicap. Because of its favorable prognosis and tendency for spontaneous regression, JXG in the larynx needs to be considered carefully with regard to whether reduction surgery and/or tracheotomy are necessary, and thus precise diagnosis is required.

Histiocytic sarcoma simulating immune thrombocytopenic purpura

Dear Editor, A 64-year-old woman presented with isolated thrombocytopenia and was diagnosed to have immune thrombocytopenic purpura (ITP). There was a slow but relentless deterioration of her platelet count. Two years later, she was found to have splenomegaly and a platelet count of 23 × 109/L. Positron emission tomography/computed tomography showed multiple mildly hypermetabolic masses in the spleen and liver. Splenectomy and liver biopsy were performed. The spleen showed sinusoidal infiltration by large tumour cells with abundant foamy cytoplasm and active erythrophagocytosis. Tumour cells were positive for the histiocytic markers lysozyme, CD68 and CD163. Liver was also involved. Features were consistent with histiocytic sarcoma. Marrow examination showed the presence of large histiocytic cells with haemophagocytosis (Fig. 1a), which were CD68 positive (Fig. 1b). Haematopoiesis appeared otherwise normal. Her platelet count recovered briefly after splenectomy, but soon deteriorated to <10 × 109/L, which was associated with frequent and distressing bleeding. Several chemotherapeutic regimens comprising cyclophosphamide, doxorubicin, vincristine, etoposide, cytarabine, bleomycin, methrotrexate, gemcitabine, cisplatin and prednisolone failed to alleviate the severe thrombocytopenia. Reassessment marrow examination showed the persistence of abnormal histiocytic cells, some reaching enormous sizes (Fig. 1c). She was finally started on the thrombopoietin mimetic eltrombopag (50 mg/day). The platelet count responded within a fortnight, rising to about 40 × 109/L. Since then, she had remained asymptomatic with stable platelet counts while on eltrombopag. Fig. 1 Histiocytic sarcoma infiltrating the bone marrow. a Histiocytic cell showing haemophagocytosis. b Immunohistochemical staining showing infiltration of the marrow by CD68-positive cells (immunoperoxidase). c Enormous histiocyte showing active haemophagocytosis ... This patient initially presented with what appeared to be ITP. However, the gradual deterioration and the finding of a splenomegaly prompted further investigations [1], which showed an infiltrative lesion in the spleen and liver. With the diagnosis of histiocytic sarcoma, marrow examination was performed, showing histiocytic infiltration. Therefore, the thrombocytopenia was attributed to marrow infiltration, although the extremely low platelet counts were incongruent with the preserved haematopoiesis. Intensive chemotherapy did not improve the platelet count or decrease the histiocytes. We surmised that the thrombocytopenia might instead be due to haemophagocytosis, and therefore after all was similar to ITP in that excessive destruction of platelets was the pathogenetic mechanism. She was treated with eltrombopag as for ITP refractory to splenectomy [2], and achieved a satisfactory response. Although eltrombopag cannot be expected to impact on the histiocytic sarcoma, it has significantly improved the quality of life of the patient and relieved her of severe thrombocytopenia and bleeding. Finally, although bone marrow examination may not be recommended for the majority of patients presenting with features characteristic of ITP [1], the judicious performance of this procedure may be necessary in patients with atypical features, particularly in the elderly.

Bilateral intraventricular mass in a child

An 8-year-old boy presented with a history of holocranial headache for 4 weeks which was mild to moderate in intensity. There was history of intermittent vomiting for the same duration. The patient was drowsy for the last 10 days. Non-contrast CT scan of head showed a space-occupying lesion in lateral ventricles with hydrocephalus. MRI showed a large lobulated well-defined heterogeneous mass lesion filling up both lateral ventricles at the posterior and temporal horns, bilaterally reaching up to the level of the foramen magnum. The mass lesion was hypointense on T2-weighted images (T2W) and isointense to grey mater on T1W images. On contrast images there was intense homogeneous enhancement seen at the lesion (Fig. 1). Right fronto-temporo-parietal craniotomy with decompression of the tumor was performed. Perioperatively, the tumor was yellowish in color, firm, not able to be aspirated and minimally vascular. Clinical diagnosis of choroid plexus tumor was considered.

Unusual Presentation of a Rare Cancer: Histiocytic Sarcoma in the Brain 16 Years After Treatment for Acute Lymphoblastic Leukemia

Histiocytic sarcoma (HS) is a very rare hematopoietic neoplasm that has been reported in association with other hematological malignancies. Presentation of HS in the central nervous system is even less common. Diagnosis of HS requires the presence of histiocytic markers and the systematic exclusion of markers of other cell lineages. Primary HS central nervous system tumors are aggressive and generally have poor outcomes. There are no standard treatment guidelines due to lack of clinical trials and a limited number of case reports. Here we present a unique case with two primary histiocytic lesions in the brain, refractory to systemic and radiation therapies, that developed after being treated for T-cell acute lymphoblastic leukemia 16 years prior.

Primitive myxoid mesenchymal tumor of infancy: Report of two cases and review of the literature

Primitive myxoid mesenchymal tumor of infancy is a recently recognized soft tissue tumor with only a few cases reported. Here, we reported another two cases of the lesion, a 5-month-old boy presenting with a soft tissue mass in the neck region that recurred 2 months later and a 3-day-old girl with a congenital superficial dorsal lumbar mass that extended to the spinal canal 1 month later. They shared similar histological patterns, such as unusual diffuse myxoid background, delicate vascular network, small cystic spaces, low to moderate cellularity, and primitive mesenchymal tumor cells. Immunohistochemically, the tumor cells showed positive for vimentin, CD99, CD117 and nestin, negative for myoid, lipoblastic, histiocytic, and neural markers. In conclusion, primitive myxoid mesenchymal tumor of infancy is a distinctive entity with its own clinical pathological features. Expression of CD99, CD117 and nestin may be consistent with the primitive nature of the tumor and may serve as ancillary markers for differential diagnosis from the other infantile tumors.

Juvenile xanthogranulomas of the nervous system: A report of two cases and review of the literature

Juvenile xanthogranulomas (JXG) are uncommon non-Langerhans cell histiocytic proliferations which arise most often in children. While most cases present as solitary cutaneous lesions, occasional cases involve extracutaneous sites. Rare examples of JXGs have been reported involving all levels of the neuroaxis. We present two cases of JXGs involving the nervous system, and review the literature. The first patient was a 14-year-old female with headaches and a mass involving the left trigeminal nerve; pathologic examination showed a JXG. At 11 months follow-up, after administration of systemic chemotherapy, the patient remained stable with residual tumor. The second patient was a 15-year-old female with leg weakness and numbness, who underwent complete surgical resection of a dural JXG. At eight months follow-up, she showed no evidence of tumor, and was able to walk without difficulty. Review of the literature revealed 38 previously published reports of JXGs involving the nervous system. The CNS was involved in the majority (75%) of cases. The clinical characteristics of JXGs arising in the CNS varied significantly from cases in the peripheral nervous system (PNS); CNS tumors occurred in younger patients, more often males, and were more likely to be associated with concurrent cutaneous and extra-nervous systemic lesions. The clinical outcomes were similar for CNS and PNS lesions, with the caveat that all three lethal JXGs occurred in the CNS. The clinical and radiologic presentation of JXGs is nonspecific, thus necessitating biopsy and pathologic examination to arrive at the diagnosis. The pathologic differential diagnosis includes a heterogeneous group of histiocytic proliferations; immunostaining for histiocytic markers CD68, factor XIIIa, and Fascin, and the absence of Birbeck granules and CD1a immunoexpression suggests the diagnosis of JXG. In many cases, total surgical resection is curative. However, some cases will require additional chemotherapy and/or radiotherapy.

Pleomorphic carcinoma of the breast with expression of macrophage markers: Report of two cases

Pleomorphic ductal carcinoma of the breast is a rare variant included in the morphological group of infiltrating ductal carcinoma. The pleomorphic carcinoma is composed predominantly of epithelial and multinucleated tumor giant cells. We report here two cases presenting a lesion composed microscopically of a proliferation of large pleomorphic cells with a predominance of multinucleated giant cells. These lesions were negative for estrogen receptor, progesterone receptor and Her2-neu (triple-negative phenotype). Basal markers (cytokeratin 5/6, cytokeratin 17 and epidermal growth factor receptor [EGFR]) were present, accompanied by the presence of histiocyte marker CD163 in most neoplastic giant cells. High-grade pleomorphic breast carcinomas with the triple-negative phenotype and expression of basal markers might be included in the basal subtype. This is the first report about the co-expression of macrophage marker CD163, with tumor (P53) or epithelial markers (CAM5.2), as indicated by double immunohistochemistry in pleomorphic ductal carcinoma of the breast.

Juvenile Xanthogranuloma Presenting as Infiltrative Conjunctival Mass in an Adult

To report on the clinical and histopathologic findings of a conjunctival juvenile xanthogranuloma in an adult. Case report and literature review. A 43-year-old man developed 2 yellowish conjunctival lumps not associated with other ocular or systemic findings. A half-corneal diameter-sized main mass was located at the 2-o'clock position, and the other 1/8-corneal diameter-sized mass was located at the 4-o'clock position. The masses were vitelliform in appearance with poor supplying vessels. The masses were removed en bloc by conjunctivectomy. Cryotherapy was done along the excision margin. Histopathologic examination revealed dense infiltration by histiocytes with background of multiple lymphocytes and Touton giant cells. Immunohistochemical staining was positive for CD3 (T-cell marker) and CD68 (histiocytic marker) but negative for CD1a and S-100 (Langerhans cell marker). Juvenile xanthogranuloma may present as conjunctival infiltrative masses in adults. Immunophenotyping is helpful in differentiating it from a more malignant lesion.

Limited Value of Immunohisto-chemistry in Diagnostic Differentiation Between Clear Cell Atypical Fibroxanthoma and Distinctive Clear Cell Mesenchymal Neoplasm With Atypical Features

To the Editor: Recently, we observed a polypoid lesion with surface ulceration (Fig. 1) on the right cheek of a 78-year-old man. The tumor was an intradermal neoplasm which involved only the superficial dermis and which was associated with a lateral collarette. The lesion consisted of large polygonal clear cells with bizarre nuclei (Fig. 1). Immunostains for pan-keratin, S100 protein, HMB45, and C-KIT were negative. The neoplastic cells showed diffuse cytoplasmic staining for vimentin, CD68, muscle-specific actin, α1-antitrypsin, and diffuse membranous staining for CD99 and CD10 (Fig. 2C–F). Mitoses were rare (less than 1 × 25 hpf). Proliferation index valuated with Mib-1 antibody was 25%. periodic acid-Schiff stain failed to demonstrate glycogen in tumor cells. Our histological diagnosis was “Clear cell cutaneous neoplasm suggestive for atypical fibroxanthoma (AFX) or distinctive clear cell mesenchymal neoplasm (DCCMN) with atypical features.” Dermal clear cell tumors comprised primarily clear cell adnexal lesions, balloon cell melanocytic lesions, metastatic clear cell carcinomas, clear cell AFXs, and DCCMNs. Histologically clear cell AFX showed a proliferative pattern similar to DCCMN. The differential diagnosis between these tumors is problematic. DCCMN was first described by Lazar and Fletcher1 as a dermal tumor composed of an homogeneous population of clear cells believed to be of mesenchymal origin. All 5 cases were present in the lower extremities. One case exhibited atypical features in the form of nuclear pleomorphism and mitotic activity. Immunohistochemical staining showed the neoplastic cells to be consistently positive for NKI-C3 and to be variably positive for CD68 and vimentin. McAlhany and LeBoit2 described 5 similar lesions with strong positivity for NK1-C3 and nuclear MiTF staining. CD68 and S100 protein were variable positive. Four cases showed focal HMB-45 expression. These authors2 believed that DCCMN might be related to “PEComas”. Later, Gavino et al3 described one case of DCCMN arising in the scalp with similar histological features to case 5 described in Lazar and Fletcher'series.1 Gavino et al3 considered these atypical features to be sufficient to justify a new pathological entity defined “atypical DCCMN”. It is evident that 2 cases are too few to permit the definition of a new histopathological entity. Gavino et al3 believed that these neoplasms could be distinguished from clear cell AFX might because “atypical DCCMN” showed to stain positively with NKI-C3 and vimentin and failed to stain with the histiocytic (CD68 and CD163), melanocytic, epithelial, and lymphoid markers. Positivity for NKI-C3, CD68, and vimentin and negativity for melanocytic, epithelial, and lymphoid markers are considered typical immunohistochemical markers for DCCMN by Lazar and Fletcher.1 The presence of NKI-C3 in 11 of 12 AFX reported by Chan et al,4 the positivity for HMB-45 and S100 protein in 4 of 5 cases described by McAlhany and LeBoit,2 the negativity for CD68 in the atypical DCCMN of Gavino et al,3 the HMB-45 expression in a clear cell AFX,5 the variable expression of muscle immunohistochemical markers in reported clear cell AFXs (Table 1)5–12 demonstrated that immunohistochemical pattern should not be used as diagnostic criteria in the differentiation between atypical AFX and DCCMN. The precise line of differentiation and the histogenesis of clear cells remain uncertain. The histological pattern may be indistinguishable. Clinical presentation (location in sun-damaged areas of the head and neck in an elderly individual) may support a histopathological diagnosis of clear cell AFX, but this tumor has been reported in the extremities. Consequently the origin site should not be considered as a definitive diagnostic criteria. It is possible that 2 neoplasms may be an unique pathological entity and future studies may elucidate the different immunohistochemical pattern. We prefer the denomination of “clear cell cutaneous neoplasm suggestive for AFX or DCCMN with atypical features”. We specify in the comment of histological report that clear cell adnexal tumors, balloon melanocytic lesions, clear cell metastatic carcinomas, and xantomatous lesions has been excluded. The immunohistochemical pattern, the presence of atypical features, and the number of mitosis per hpf are reported. The lesion should be considered of unknown biological potential.1FIGURE 1: Scanning magnification shows an exophytic polypoid lesion with surface ulceration (insert), composed by neoplastic cells of varying sizes with clear to reticulated cytoplasm and enlarged, vesicular nuclei with prominent nucleoli. Markedly atypical neoplastic cells are scattered throughout the nodule.FIGURE 2: Immunohistochemical stains show diffuse cytoplasmic positivity for vimentin (C), muscle specific actin (D), and diffuse membranous staining for CD99 (E), and CD10 (F).TABLE 1: Review of Immunohistochemical Pattern of Clear Cell Atypical Fibroxantoma

CD163 versus CD68 in tumor associated macrophages of classical hodgkin lymphoma

Classical Hodgkin lymphoma (CHL) is a B-cell lymphoproliferative disorder with a relatively good prognosis. A small but significant percentage of patients, however, will respond poorly to therapy. A recent gene expression profiling study has identified a macrophage signature which has been correlated with primary treatment failure, and immunohistochemical tissue microarray for CD68 was shown to reflect the gene signature as a potentially clinically useful marker to predict adverse prognosis.We examined 44 cases of CHL, mostly nodular sclerosis subtype, in which the immunohistochemical stains for the histiocytic markers CD68 and CD163 were performed. The staining intensity was graded for each stain (< 5, 5-25, and > 25 percent of cells positive in the Hodgkin cell (HC) rich nodules) and background staining characteristics were recorded.CD163 staining was lower than CD68 in HC rich nodules, with lower background staining (p 0.03). There was no significant difference between either CD68 or CD163 and disease recurrence in a subset (N = 41) of cases.In conclusion, we demonstrate that CD163 staining is lower than CD68, with less non-specific staining of background inflammatory cells and Hodgkin cells, therefore is a better marker for tumor associated macrophages. However, we did not identify a correlation between staining for CD68 or CD163 and recurrence of disease.Virtual slidesThe virtual slide(s) for this article can be found here:http://www.diagnosticpathology.diagnomx.eu/vs/1460518258831620

Xanthogranulomatous Inflammation of the Female Genital Tract: Report of Three Cases

Purpose and Methods: This is a series of three cases diagnosed with xanthogranulomatous inflammation of the female genital with emphasis on the etiology, clinical-pathologic features and biological behavior. Clinical, pathologic, radiologic and follow up data are reported.Results: The three cases of Xanthogranulomatous inflammation of the female genital tract are the followings: 1) one case affecting the endometrium, 2) one case affecting the fallopian tube, and 3) one case confined to the ovary. The patient's age was 37, 22 and 62 year-old, respectively. Histologic examination revealed extensive infiltration of foamy histiocytes admixed with variable amount of inflammatory cells. The later include plasma cells, lymphocytes, and occasional multinucleated giant cells. Immunohistochemistry showed positive staining for CD68, a histiocytic marker, in foamy histiocytes, CD3, a T cell marker, and CD20, a B cell marker, in the background lymphocytes. The plasma cells were polyclonal with expression of both κ and λ light chains.Conclusion: Xanthogranulomatous inflammation of the female genital tract is an unusual lesion, and clinically forms mass- like lesion in the pelvic cavity that invades the surrounding tissues, which may mimic the tumor clinically and by imaging.

An Unusual Pulmonary Mass With Mediastinal Invasion and Multiple Intrapulmonary Nodules in a 52-Year-Old Man

An Unusual Pulmonary Mass With Mediastinal Invasion and Multiple Intrapulmonary Nodules in a 52-Year-Old Man

Littoral cell angiomas of the spleen associated with solid pseudopapillary tumor of the pancreas

Littoral cell angiomas (LCA) of the spleen are vascular tumors of unknown etiology arising from the littoral cells of the splenic red pulp sinuses. Usually a benign and incidental finding, LCA have been repeatedly reported in association with a variety of visceral malignancies and hold the potential for dissemination per se. We encountered a case of a 30 year old female who was diagnosed with solid pseudopapillary tumor of the head and distal pancreas by fine needle aspiration cytology. A distal pancreatectomy with splenectomy was performed in addition to a pylorus-preserving Whipple's procedure and cholecystectomy. Histopathological examination confirmed solid pseudopapillary tumor of the pancreas and showed multiple well-circumscribed anastomosing vascular channels in the spleen. The diagnosis of LCA of the spleen was confirmed by immunohistochemistry that revealed co-expression of endothelial cell marker, CD31 and CD34, along with histiocytic marker, CD68 by the vascular lining cells. LCA has been previously reported in association with colorectal and pancreatic adenocarcinoma, malignant lymphoma, myelodysplasia and autoimmune disorders. We report the first case of LCA associated with solid pseudopapillary tumor of the pancreas.

The Langerhans cell histiocytosis

The Langerhans cell histiocytosis

Histiocytic Sarcoma and Tumor Lysis Syndrome: An Unexpected Sequela of Neck mass

1. Vos, et al. “Histiocytic sarcoma: a study of five cases including the histiocyte marker CD163” Modern pathology. Jan 2005 (18)p693-704 2. De Vos, FYFL, Gerding M, Ardens J, Wegmen J. Histiocytic sarcoma localised in the thyroid—a case report. Ann Hematol 2008;87(8): 681682. 3. World Health Organization Classification of Tumours (2001) Pathology and genetics of tumours of hematopoietic and lymphoid tissue. International Agency for Research on Cancer (IARC), Lyon, pp 274–289 . 4. Cairo M, Bishop M. Tumor lysis syndrome: new therapeutic strategies and classification. British J Haematology 2004;127:3-11. 5. Gokmen Akoz A, Yildirim N, Engin H, et al. An unusual case of spontaneous acute tumor lysis syndrome associated with acute lymphoblastic leukemia: A case report and review of the literature. Acto Oncol 2007; 46(8):1190-2. The patient continued to rapidly deteriorate, developing disseminated intravascular coagulation (DIC), multi-system organ failure, and decreased neurologic function. The patient was pronounced dead early the following morning, just two days after admission. Her final pathology was consistent with histiocytic sarcoma. Histology revealed sheets of large atypical cells with abundant eosinophilic cytoplasm, large irregular nuclei and prominent nucleoli (Figure 4). Staining was positive for CD4, CD45 and CD68, and negative for B and T cell markers as well as keratin and HMB-45 (Figures 5,6). Her cause of death was felt to be spontaneous tumor lysis syndrome caused by massive tumor burden. Approximately 1/3 of cases present in lymph nodes, 1/3 in skin and 1/3 in extranodal sites, with the GI tract being most common.3 Treatment of histiocytic sarcoma consists of wide local excision when possible with adjuvant radiotherapy. Chemotherapeutic regimens have been proposed without evidence-based data.2 Patients have a very poor prognosis with poor response to therapy except in the case of very limited disease where complete excision is possible.1,2 Our case is unique in that histiocytic sarcoma does not often present as a neck mass and therefore does not typically enter into the differential diagnosis of otolaryngologists.