NF1 mutation and TUBB3 amplification in gastric histiocytic sarcoma: a case report and literature review.

Histiocytic sarcoma is a rare neoplasm of mature histiocytes with an aggressive clinical course and poor response to treatment. Primary gastric histiocytic sarcoma is rarer and just reported sporadically.Histiocytic sarcoma is a rare neoplasm of mature histiocytes with an aggressive clinical course and poor response to treatment. Primary gastric histiocytic sarcoma is rarer and just reported sporadically. A case of a 71-year-old female admitted with a one-year history of upper abdominal pain exacerbated after meals. After CT scans revealed a bulged mass at the lesser curvature of the gastric body, the patient underwent endoscopic submucosal dissection. Microscopically, non-cohesive neoplastic cells diffusely infiltrated lamina propria and submucosa, and diffusely expressed LCA, CD4, CD163, CD68 (KP1), Cyclin D1, Lysozyme, and Vimentin. PD-L1 (22CS) expression evaluated as CPS 60. The final pathological diagnosis was gastric histiocytic sarcoma. Subsequently, next-generation sequencing identified a nonsense mutation in exon 21 of NF1 gene [c.2446C > T (p.R816*)] and the TUBB3 gene amplification (copy number: 4.55). The patient refused further treatment and died of the tumor half a year later. This case broadens the spectrum of differential diagnosis of gastric cancer and emphasizes the value of immunohistochemical and molecular tests in the accurate diagnosis of histiocytic sarcoma. Furthermore, we performed literature review of 11 cases of gastric histiocytic sarcoma so as to strengthen the understanding of the clinicopathologic features, treatment, and prognosis.

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 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.

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.

[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 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.

Histiocytic sarcoma of the parotid gland region.

Histiocytic sarcoma (HS) is a malignant neoplasm showing the morphological and immunophenotypic features of mature histiocytes. Reported herein is a case of HS of the parotid gland region. A 53-year-old woman noticed a swelling of the right preauricular area. Preoperative fine needle aspiration cytology showed an admixture of pleomorphic atypical cells and mature lymphocytes. She underwent total parotidectomy. Grossly, the tumor was located at the parotid gland to subcutaneous tissue, and showed infiltrative growth with massive necrosis and hemorrhage. Microscopically, the tumor was composed of marked pleomorphic cells with eosinophilic cytoplasm. Bizarre multinuclear giant cells were scattered and intermingled. Tumor cells were positive for CD68 (KP-1 and PG-M1), CD163, S-100 protein, CD1a, CD4 and CD31, but negative for CD3, CD20, CD21, CD79a, DEC205 and langerin, immunohistochemically. Monoclonal proliferation of B cells was not confirmed on polymerase chain reaction for IgH. The patient had recurrent lesions in the pelvis and stomach 5 months after parotidectomy and died of the disease 10 months after the operation.

[A rare cutaneous congenital sarcoma: plexiform fibrohistiocytic tumor]. / Un sarcome cutané congénital rare: la tumeur fibrohistiocytaire plexiforme.

We report the unusual case of a three-year-old girl which presented since birth a pigmented tumor of the left side 0.5cm in diameter. Surgical removal was decided given the hypothesis of a congenital naevo-cellular naevus. The histological study ended with the diagnosis of plexiform fibrohistiocytic tumor (PFHT). Two other congenital PFHT have been reported until now. PFHT is a rare mesenchymal neoplasm of intermediate malignancy, first reported by Enzinger and Zhang in 1988 which may be difficult to diagnose, because of its low frequency, particulary in congenital cases. It is important to distinguish it from others childhood cutaneous tumors (particulary plexiform neurofibroma, cellular neurothekeoma, infantile myofibromatosis, and fibrous hamartoma). The tumor has a high local recurrence rate and complete surgical resection of the tumor, with wider margins, is required.

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.

[Histiocytic sarcoma: a clinicopathologic study of 6 cases].

OBJECTIVE: To study the morphologic features, immunophenotypes, differential diagnoses and prognosis of histiocytic sarcoma (HS). METHODS: The clinical and pathologic findings of 6 cases of HS were reviewed. Immunohistochemical assay (Elivision staining) was also performed. Follow-up information was available in 4 patients. RESULTS: There were altogether 3 males and 3 females. The age of patients ranged from 12 to 81 years old (median = 54.6 years). The sites of involvement included lymph node (number = 2 cases) and skin or soft tissue (number = 4 cases). The tumor was composed of sheets of large epithelioid cells with abundant eosinophilic cytoplasm, oval to irregular nuclei, vesicular chromatin and large nucleoli. Binucleated form was not uncommon. Two of the cases showed increased pleomorphism with multinucleated tumor giant cell formation. Focal cytoplasmic with foamy appearance was identified in 3 cases. One case demonstrated foci of spindly sarcomatoid appearance. Hemophagocytosis was identified in 2 cases. Mitotic figures were readily identified. The tumor cells were often accompanied by various numbers of inflammatory cells. Immunohistochemical study showed that all cases were diffusely positive for leukocyte common antigen, CD4, CD68 and CD163. Four of the 5 cases studied also expressed lysozyme. Amongst the 4 patients with follow-up information available, 3 died of the disease at 6 to 11 months interval after diagnosis. One patient, whose lesion was localized at the skin and soft tissue, survived for 3 years, with no evidence of tumor recurrence. CONCLUSIONS: Accurate diagnosis of the HS is based on the combination of morphologic examination and immunohistochemical assay. HS often presents with clinically advanced disease and pursues an aggressive clinical course, with a poor response to therapy. However, a subset of cases presenting with clinically localized lesion may carry a relatively favorable long-term outcome.

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.

Thoracic omentalization for long-term management of neoplastic pleural effusion in a cat.

CASE DESCRIPTION: An 11-year-old neutered female domestic longhair cat was evaluated because of a 1-week history of progressive dyspnea, signs of depression, and loss of appetite. A histiocytic sarcoma had been excised from the mammary gland 6 weeks earlier. CLINICAL FINDINGS: Physical examination findings were consistent with pleural effusion, and thoracic and abdominal radiography and ultrasonography revealed pleural effusion, a thoracic mass involving the aorta and pulmonary artery, and a caudal abdominal mass that most likely represented enlarged iliac lymph nodes. Cytologic examination of the pleural fluid and fine-needle aspirates from the iliac and right popliteal lymph nodes revealed abundant cells with neoplastic characteristics of indeterminate origin. The clinical diagnosis was generalized malignant neoplasia. TREATMENT AND OUTCOME: Pleural drainage was necessary every 5 to 6 days. Exploratory thoracotomy and biopsy of the mass were recommended for better characterization of the thoracic disease. Simultaneously, palliative treatment by advancement of the omentum into the thorax was performed. A final diagnosis of disseminated histiocytic sarcoma was made, and treatment with doxorubicin was begun after surgery. During the 13 months after surgery, the cat was free from signs of respiratory tract disease and had normal activity levels with good exercise tolerance. Fifteen months after surgery, the cat's clinical condition worsened and the cat died. CLINICAL RELEVANCE: Findings suggested that thoracic omentalization may be considered for palliative treatment of cats with refractory neoplastic pleural effusion when frequent thoracocentesis is necessary and other treatments are not suitable.

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.

Histiocytic sarcoma of the stifle in a dog with cranial cruciate ligament failure and TPLO treatment.

A 10-year-old spayed female Golden Retriever was admitted with chronic lameness of the right hind limb. A tibial plateau leveling osteotomy (TPLO) had been performed on this leg approximately three years previously. A progressively growing soft tissue mass, affecting the right stifle, previously treated with TPLO was biopsied and found to be a histiocytic sarcoma. Previously proposed links between the development of neoplasia in the stifle region and the presence of chronic synovitis, osteotomy, orthopaedic implants, and specifically the Slocum TPLO plate, are briefly discussed.

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.

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.