[Pulmonary histiocytic sarcoma]. / A tüdo histiocytás sarcomája.

Histiocytic sarcoma is an uncommon hematological malignancy. Its occurrence in the lung is very rare. Due to the small number of cases and the clinical and pathological features of the disease, the diagnosis can be challenging. Its optimal treatment is not yet known, in locally confined cases - depending on the location and size - surgical removal is part of complex oncotherapy. We report the case of a 52-year-old man with a tumor of central localization in the left lung. Pulmonectomy was performed. Histology verified histiocytic sarcoma of the lung. An overview of clinical features of the entity is presented in connection with our case report. Orv Heti. 2023; 164(34): 1350-1357.

Histiocytic sarcoma of the palate: a case report.

Histiocytic sarcoma is a malignant proliferation of cells that exhibit morphological and immunophenotypic features of mature histiocytes. Owing to its rarity, its clinical features and standard treatment have not yet been established. This report describes a case of histiocytic sarcoma of the palate that developed in a 76-year-old man, the first report of an intraoral histiocytic sarcoma. An extended resection was performed; however, establishing the excision line was extremely difficult because assessing the tumour boundary on imaging was challenging and the tumour underwent dynamic gross morphological changes following biopsy. Complete resection is required to obtain a favourable prognosis for high-grade tumours with indistinct borders. In this case, an intraoperative rapid examination with frozen section analysis was performed along the planned excision line to completely resect the tumours exhibiting such behaviour. At 28 months postoperatively, the patient demonstrated no recurrence or metastasis; however, he is under careful monitoring.

A rare case of histiocytic proliferative disease in a cat.

A 7-year-old intact female domestic medium hair cat was examined at a veterinary clinic for a scabbed nodule over the right shoulder. Multiple nodules recurred at the same site after the first surgical excision, and a second surgical excision was performed. Histopathology demonstrated high-mitotic-rate neoplastic cells and therefore a histiocytic proliferative disease was initially suspected. The condition progressed rapidly within a 5-month period and the cat was euthanized due to sudden onset of severe dyspnea. Necropsy showed diffuse metastatic nodules in the lungs, confirming a histiocytic proliferative disease, with histiocytic sarcoma being the most likely differential diagnosis.

Un cas rare de maladie histiocytaire proliférative chez un chat. Une chatte domestique á poil moyen intacte de 7 ans a été examinée dans une clinique vétérinaire pour un nodule croûteux sur l'épaule droite. Plusieurs nodules sont réapparus au même site après la première excision chirurgicale, et une deuxième excision chirurgicale a été réalisée. L'histopathologie a mis en évidence des cellules néoplasiques á taux mitotique élevé et, par conséquent, une maladie proliférative histiocytaire a été initialement suspectée. L'état a progressé rapidement en l'espace de 5 mois et le chat a été euthanasié en raison de l'apparition soudaine d'une dyspnée sévère. L'autopsie a montré des nodules métastatiques diffus dans les poumons, confirmant une maladie proliférative histiocytaire, le sarcome histiocytaire étant le diagnostic différentiel le plus probable.(Traduit par Dr Serge Messier).

Histiocytic Sarcoma with Invasive Urothelial Carcinoma of Urinary Bladder.

We report a rare case of histiocytic sarcoma in association with invasive urothelial carcinoma involving the urinary bladder. A 67-year male patient, who presented with a complaint of macroscopic hematuria, was found to have a mass on urinary system ultrasonography. Abdominal magnetic resonance imaging was performed to evaluate the mass, which showed a 52×24 mm mass on the posterior wall of the bladder. The cystoscopic examination revealed two suspicious areas in close proximity to one another with solid-papillary character. The tissue samples were collected by means of transurethral resection. The evaluation of these samples revealed two distinct neoplastic patterns. The areas of invasive urothelial carcinoma infiltrating lamina propria were noted on the surface in addition to diffuse sheets of large cells with hyperchromatic nuclei and ample clear cytoplasm, with a patternless pattern among small lymphocytes in myxoid background in the lamina propria. The positive reactions were observed in these areas with CD45, fascin, and CD68, a histiocytic marker. The histopathological diagnosis was histiocytic sarcoma in combination with invasive urothelial carcinoma. Histiocytic sarcoma may mimic several other malignant lesions, and only immunohistochemistry can identify this tumour to allow correct treatment. We present this rare case to emphasise that this phenomenon should be considered in unusual tumors and sites. Key Words: Urinary bladder, Histiocytic sarcoma, Invasive urothelial carcinoma.

An advanced case of gastric histiocytic sarcoma treated with chemotherapy and gastrectomy: a case report and review of literature.

Histiocytic sarcoma is a relatively new disease category and the gastrointestinal origin is sporadic. We report a case of a 74-year-old woman who underwent chemotherapy and proximal gastrectomy for extremely rare, advanced gastric histiocytic sarcoma. The resected specimen was subjected to numerous immunostainings to meet the diagnostic criteria of histiocytic sarcoma and was positive for the histiocyte markers' cluster of differentiation 68 and lysozyme. The markers of Langerhans cells, follicular dendritic cells, and myelocyte were all negative. Six reports of surgical resection of histiocytic sarcoma originating in the stomach exist, including our case. We reviewed the clinical course and the histological and immunohistochemical diagnostic features of surgically resected gastric histiocytic sarcoma.

Clinical outcomes in dogs with localized splenic histiocytic sarcoma treated with splenectomy with or without adjuvant chemotherapy.

BACKGROUND: Localized splenic histiocytic sarcoma (HS) in dogs is a poorly understood disease, and could have longer survival times than disseminated or hemophagocytic HS. Understanding the clinical behavior of localized splenic HS can refine treatment recommendations. OBJECTIVE: To describe the clinical characteristics and outcomes of dogs with localized splenic HS. ANIMALS: Fourteen client-owned dogs with histologically confirmed splenic HS that received splenectomy. METHODS: Multi-institutional retrospective case series-medical records of dogs with splenic HS were reviewed. Dog signalment, clinicopathologic data, primary and adjuvant treatments, and outcomes were obtained. Survival data were calculated using Kaplan-Meier analysis. Dog variables such as age, weight, platelet counts were reported using descriptive statistics. The Cox proportional hazards regression method was used to determine whether potential risk factors (weight, age, albumin level, hematocrit, and platelet count) were associated with PFI. RESULTS: Median survival time for the dogs in this study was 427 days. Twelve dogs received adjuvant lomustine-based chemotherapy. Five dogs (35.7%) were suspected or confirmed to have developed metastatic disease. Eleven dogs died of disease, 1 dog died of unrelated cause, and 2 dogs were alive at final follow-up. CONCLUSIONS AND CLINICAL SIGNIFICANCE: Histiocytic sarcoma in dogs can manifest as a localized form in the spleen. Dogs with localized splenic HS treated with surgery ± chemotherapy can experience survival times over a year.

Outcome comparison between radiation therapy and surgery as primary treatment for dogs with periarticular histiocytic sarcoma: An Italian Society of Veterinary Oncology study.

Localized histiocytic sarcoma may occur as a primary lesion in periarticular tissues of large appendicular joints. Treatment options for the primary lesion include radical surgical excision, radiation therapy (RT), or both, in combination with chemotherapy for potential systemic metastases. In an effort to better characterize the time to progression (TTP) following surgical vs non-surgical approaches for periarticular histiocytic sarcoma (PAHS), a contemporary European population of affected dogs was retrospectively surveyed. Medical records were queried for newly-diagnosed PAHS cases undergoing surgery (predominantly limb amputation) or RT followed by systemic chemotherapy. Of 49 dogs, 34 underwent RT and 15 underwent surgery. All dogs received adjuvant chemotherapy. There was no statistically significant difference in TTP or overall survival between groups. The median TTP was 336 days for the operated dogs and 217 days for the irradiated dogs (P = .117). The median overall survival time was 398 days for the operated dogs and 240 days for the irradiated dogs (P = .142). On multi-variable analysis, the variables significantly associated with an increased risk of both tumour progression and tumour-related death were regional lymph node and distant metastasis at admission. Survival and local control rates following RT may be comparable to radical resection. These data may better inform shared decision-making processes between multi-disciplinary care providers and owners.

Primary cutaneous localized/clearly-outlined true histiocytic sarcoma: Two long-term follow-up cases.

Histiocytic sarcoma (HS) is a rare hematopoietic tumor that mainly involves extranodal sites, including the intestinal tract, skin, soft tissues and other organs. It is well known as an aggressive neoplasm that shows a poor response to therapy. However, a subset of patients with resectable disease has shown a favorable outcome with surgical treatment. Primary cutaneous HS is exceedingly rare and, to date, its long-term prognosis has thus not been well described. Here, we highlight two cases of primary cutaneous HS that showed long-term survival. Case 1 was a healthy 47-year-old woman who found a 12-mm tumor on her forehead. Case 2 was a 66-year-old woman, under follow up of a myxoid liposarcoma in her leg, who presented with a 25-mm tumor in her hypothenar eminence. Histologically, the tumors in both cases had a smooth outline with proliferating atypical tumor cells that showed histiocytic differentiation as revealed by immunohistochemistry with antibodies to CD68 (KP-1) and lysozyme in case 1; and CD68, lysozyme and CD163 in case 2. Tumor cells in case 1 had a monotonous appearance. After complete resection, cases 1 and 2 have survived for 10 and 4 years, respectively, without recurrence. To date, such patients are relatively long follow-up cases of survival from HS and highlight how a clear outline of the primary cutaneous HS tumor may be associated with its resectability and be an important factor in the assessment of its curability.

Clinical management and outcome of four-toed hedgehogs (Atelerix albiventris) with histiocytic sarcoma.

Four-toed hedgehogs presented bloody stool and loss of appetite. Integumental masses were observed in two of the four cases. Intraabdominal masses were observed on radiographs and ultrasonography in the remaining two cases. The masses were surgically removed from all four cases. All samples were histologically and immunohistochemically consistent with histiocytic sarcoma (HS). At the time of surgery, 3 of 4 cases had grossly recognized metastatic/disseminated lesions. Survival time was 48 days, 64 days, and 113 days, respectively, and the remaining case is currently alive (at Day 207). The present report describes the clinical management and outcome of hedgehogs that were diagnosed with HS.

Bony Histiocytic Sarcoma of the Scapula: Case Report / Sarcoma histiocítico ósseo em escápula: relato de caso

Abstract Case report of a pediatric patient with a diagnosis of bony histiocytic sarcoma of the scapula, submitted to oncologic resection (Tikhoff-Linberg type II surgery), who returned to previous activities and was disease-free after 24 months of follow-up.

Resumo Relato de caso de paciente pediátrico com diagnóstico de sarcoma histiocítico ósseo em escápula, submetido à ressecção oncológica (cirurgia de Tikhoff-Linberg tipo II), com retorno às atividades prévias e livre de doença após 24 meses de seguimento.

Histiocytic sarcoma in a child-successful management and long-term survival with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

Histiocytic sarcoma is an extremely rare tumor in children. It may occur sporadically or in association with other hematological malignancies. It arises most commonly in the lymph nodes but may occur anywhere in the body and clinical presentation is usually with advanced disease. Following tissue diagnosis and staging, management is with chemotherapy though there are no standard regimes. Surgery has been used successfully for local control. This is the first description of the use of peritonectomy and hyperthermic intraperitoneal chemotherapy to treat histiocytic sarcoma. The 4-year-old patient has been disease free for 6 years.

Retroperitoneal Bulky Histiocytic Sarcoma Successfully Treated with Induction Chemotherapy Followed by Curative Surgery.

Histiocytic sarcoma (HS) is a rare hematopoietic neoplasm. We report a patient with HS treated with induction chemotherapy followed by curative surgery. A 50-year-old man was referred to our hospital because of a retroperitoneal tumor. A computed tomography scan revealed a bulky retroperitoneal mass, infiltrating the surrounding organ. An excisional biopsy confirmed the diagnosis of HS. The tumor shrunk after multidrug chemotherapy. However, positron emission tomography showed uptake of fludeoxyglucose in the residual tumor. He underwent right nephrectomy to remove the tumor. Pathological examination showed complete response. Surgery combined with induction chemotherapy may be an effective way to manage HS.

Histiocytic sarcoma: New insights into FNA cytomorphology and molecular characteristics.

BACKGROUND: Histiocytic sarcoma (HS) is a rare malignant neoplasm showing morphologic and immunophenotypic features of histiocytes. Molecular characteristics of HS and fine-needle aspiration (FNA) criteria for its diagnosis have not been established. METHODS: A case series of HS in 8 FNA samples from 6 patients was reviewed along with histopathologic and clinical data. Immunohistochemistry was performed on cell blocks (3 cases), core biopsies (5 cases), and surgical specimens (4 cases). Targeted-exome next-generation sequencing (NGS) was performed on surgical resection specimens in 4 cases. RESULTS: Four patients had a known history of hematolymphoid malignancy. Cytomorphologic features included variably cellular smears composed of large epithelioid cells with reniform nuclei and abundant vacuolated cytoplasm, in an inflammatory background, with occasional cytophagocytosis and lymphoglandular bodies. Marked pleomorphism, multinucleated monster cells, and binucleated histiocytoid cells with partially overlapping, eccentrically placed nuclei resembling Pac-Man were common. Most cases expressed histiocytic markers CD68 (6 of 7 cases), CD163 (5 of 5 cases), and PU.1 (3 of 4 cases). In 3 cases, NGS analysis revealed alterations in lysine methyltransferase 2D (KMT2D)/mixed-lineage leukemia 2 (MLL2), a gene involved in chromatin regulation and previously implicated in the pathogenesis of follicular lymphoma. CONCLUSIONS: Although diagnosing HS with FNA alone is extremely challenging, the presence of pleomorphic and epithelioid large cells with binucleation and/or multinucleation in an inflammatory background should prompt the diagnosis of HS with judicious use of confirmatory histiocytic lineage markers. The detection of recurrent KMT2D/MLL2 alterations implicates epigenetic regulation in the pathogenesis of HS and supports the notion of transdifferentiation from a genetically similar but phenotypically distinct tumor of a different lineage. Cancer Cytopathol 2017;125:604-14. © 2017 American Cancer Society.

Infection or Glioma? The False Dilemma of Primary Central Nervous System Histiocytic Sarcoma.

BACKGROUND: Primary central nervous system (CNS) histiocytic sarcoma is an extremely rare lymphoproliferative disorder that affects the CNS and behaves aggressively. Only 27 cases of primary CNS histiocytic sarcoma have been reported. The paucity of literature on this entity has made diagnosis and treatment difficult both for the surgeon and the pathologist. CASE DESCRIPTION: In this case of primary CNS histiocytic sarcoma, a middle-aged woman presented from an outside institution with a supposed cerebellar abscess. Intraoperative frozen pathology was initially interpreted as high-grade glioma; however, final pathology demonstrated histiocytic sarcoma. CONCLUSIONS: This report makes a significant contribution to the literature on this rare malignant disease by outlining a similar presentation among several cases and providing a thorough overview of existing criteria for diagnosis and management.

Sarcoma histiocítico oral en región de carcinoma epidermoide previo: a propósito de un caso / Oral histiocytic sarcoma in previous epidermoid carcinoma region: case report

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Chemotherapy for dogs with lymph node metastasis from histiocytic sarcomas.

BACKGROUND: Histiocytic sarcomas (HS) frequently metastasise, most commonly to visceral sites, but also to regional lymph nodes. Nodal metastases are associated with a poorer prognosis. This retrospective study aimed to evaluate prognostic factors, including the effect of adjuvant chemotherapy, on survival in dogs with nodal, but not systemic, metastases from HS. METHODS: Retrospective case series of 12 dogs with histologically diagnosed HS metastatic to lymph nodes treated with surgery with and without adjuvant chemotherapy. RESULTS: All dogs had histological evidence of metastasis to lymph nodes, with no clinical evidence for metastasis to viscera. Eight dogs that received chemotherapy had a median estimated survival of 219 days (range 77-1638 days); 1- and 2-year estimated survival rates were 37.7%. Median survival time for 4 dogs with nodal metastases that did not receive chemotherapy was 57 days (range 39-136 days) with none alive 1 year after surgery CONCLUSION: Survival for dogs with only regional nodal metastases from HS appeared to be improved by adjuvant chemotherapy.

Histiocytic Sarcoma Originating in the Lung in a 16-Year-Old Male.

We report a 16-year-old male with histiocytic sarcoma (HS) originating in the lung. Partial resection of the lung was performed for a 3-cm mass with a clear boundary detected in the right inferior pulmonary lobe on a health checkup. Histologically, the tumor infiltrated into the surrounding tissue, and was comprised of spindle cells, mainly, and foam cells accompanied by mild nuclear atypia. The tumor cells were immunohistochemically positive for CD68 and CD163, indicating histiocytic lineage and the MIB-1-positive rate was low. Spindle cell morphology of HS is quite rare and only 3 cases of pulmonary HS have previously been reported.

A Case of G-CSF-Producing Histiocytic Sarcoma of the Stomach.

No reports have been published to date regarding primary gastric granulocyte colony-stimulating factor (G-CSF)-producing histiocytic sarcoma. We encountered a case of primary gastric histiocytic sarcoma that also fulfilled the criteria for a G-CSF-producing tumor. A 75-year-old man was diagnosed with gastric cancer with poorly differentiated adenocarcinoma. The patient's white blood cell count was elevated to 20,700/µL, and the G-CSF level was elevated to 380 pg/mL. A computed tomography scan showed hepatic infiltration; therefore, a preoperative diagnosis of T4 (liver) N2H0M0 cStage IV gastric cancer was made, and surgery was performed. No. 11d lymphatic metastasis was noted, resulting in invasion of the pancreatic tail, and combined resection of the liver, pancreas, and spleen was conducted with complete gastrectomy. The results of hematoxylin-eosin and immunohistochemical staining were subsequently assessed. On discharge, the G-CSF level had fallen to 22.7 pg/mL. Currently, the patient is still alive and has experienced no recurrence approximately 4 years after the operation.

Histiocytic sarcoma of the bile duct.

An 80-year-old man was admitted with anorexia, a high fever and general icterus. Laboratory examinations revealed remarkable inflammation and elevation of liver/biliary enzymes. Computed tomography (CT) showed a high-density lesion in the bile duct, and endoscopic retrograde cholangiopancreatography (ERCP) disclosed an oval filling defect mimicking choledocholithiasis. Plastic stents were inserted to treat the acute cholangitis; however, the patient's symptoms recurred. An erythematous hypervascular mass obtained with a balloon catheter contained numerous pleomorphic histiocytic cells with eosinophilic cytoplasm, remarkable anisonucleosis and occasional mitoses. A diagnosis of histiocytic sarcoma (HS) was made based on the results of intensive immunohistochemistry. Monoclonal rearrangement of the IGH and TCRG genes, IGH split and IGH/BCL2 fusion was negative, although polysomy 8, 14, and 18 was detected. The patient was treated conservatively and died of the disease 20 months after the initial diagnosis. To the best of our knowledge, this is the first case of bile duct HS. This case, which involved numerical alterations of chromosomes, presented with CT and ERCP findings similar to those of choledocholithiasis.