Effects of extramedullary disease on patients with newly diagnosed multiple myeloma / 中华血液学杂志

Objective: To summarize the characteristics of patients with newly diagnosed multiple myeloma (NDMM) admitted at Ruijin Hospital affiliated to Shanghai Jiaotong University School of Medicine. We compared the clinical characteristics and prognoses among patients with non-extramedullary disease (EMD), bone-related extramedullary (EM-B) disease, and extraosseous extramedullary (EM-E) disease and further explored the effects of autologous hematopoietic stem cell transplantation (ASCT) for EMD. Methods: From January 2015 to January 2022, data of 114 patients (22%) with EMD out of 515 patients with NDMM were retrospectively analyzed; 91 (18%) and 23 (4%) patients comprised the EM-B and EM-E groups, respectively. The clinical characteristics of patients in all groups were compared with the Chi-square test. Progression-free survival (PFS) and overall survival (OS) of patients were analyzed by the Kaplan-Meier method. Independent prognostic factors were determined using multivariate Cox proportional hazard model. Results: There were no significant differences in age, gender, ISS stage, light chain, creatinine clearance, cytogenetic risk, 17p deletion, ASCT, and induction regimens among the three groups. Overall, 13% of EM-E patients had IgD-type M protein, which was significantly higher than that in EM-B patients (P=0.021). The median PFS of patients in the non-EMD, EM-B, and EM-E groups was 27.4, 23.1, and 14.0 months; the median OS was not reached, 76.8 months, and 25.6 months, respectively. The PFS (vs non-EMD, P=0.004; vs EM-B, P=0.036) and OS (vs non-EMD, P<0.001; vs EM-B, P=0.002) were significantly worse in patients with EM-E, while those were not significantly different between patients with EM-B and those with non-EMD. In the multivariate analysis, EM-E was an independent prognostic factor for OS in patients with NDMM (HR=8.779, P<0.001) and negatively impacted PFS (HR=1.874, P=0.050). In those who did not undergo ASCT, patients with EM-B had significantly worse OS than those with non-EMD (median 76.8 months vs. not reached, P=0.029). However, no significant difference was observed in the PFS and OS of patients with EM-B and those with non-EMD who underwent ASCT. Conclusions: Compared to patients with either non-EMD or EM-B, those with EM-E had the worst prognosis. EM-E was an independent risk factor for OS in patients with NDMM. ASCT can overcome the poor prognosis of EM-B.

Primary Ewing sarcoma/primitive neuroectodermal tumor of the lung

We present the autopsy findings and differential diagnosis in a 42year old male who presented with fever and rapidly progressive respiratory symptoms like breathlessness, nonproductive cough and right sided chest pain. Initial imaging workup done at our hospital revealed a large unilateral tumor with tracheal shift. While being evaluated patient developed facial puffiness, tachypnea suggestive of superior vena cava obstruction. Antemortem biopsy of lung mass was attempted twice and that suggested malignant lesion. Unfortunately, the individual had a rapid downhill course following admission. Post mortem examination was conducted that on opening the thoracic cavity revealed total replacement of right lung tissue by a necrotic growth which was deeply adherent to the rib cage. The contralateral lung as well as all other visceral organs were unremarkable grossly. Histopathology confirmed primary Ewing sarcoma of the lung. We hereby, report a rare case of primary lung Ewing sarcoma diagnosed at autopsy.

Accidente cerebro vascular visualizado en gammagrafía conTc-99m MDP, un hallazgo extraóseo inusual / Stroke in scintigraphy with Tc-99m MDP, an unusual extraosseous finding / Acidente vascular cerebral visualizado na cintilografia com Tc-99m MDP, um achado extra-sensorial incomum

Se presenta el caso de un paciente con antecedente de adenocarcinoma de próstata y diagnóstico de ACV isquémico con transformación hemorrágica. La gammagrafía ósea con Tc-99m MDP complementada con SPECT e imágenes de fusión con TC cerebral simple, realizada a los 15 días, muestra hipercaptación en la región temporal izquierda, correspondiente al ACV en etapa subaguda.

We present the case of a patient with a history of prostate adenocarcinoma with a diagnosis of ischemic stroke with hemorrhagic transformation. Bone scintigraphy with Tc-99m MDP supplemented with SPECT and fusion images with simple cerebral CT, performed at 15 days, shows uptake in the left temporal region, corresponding to stroke in subacute stage

Apresentamos o caso de um paciente com história de adenocarcinoma de próstata com diagnóstico de acidente vascular cerebral isquêmico com transformação hemorrágica. A cintilografia óssea com Tc-99m MDP suplementada com SPECT e imagens de fusão com TC cerebral simple, realizadas aos 15 dias, mostra captação na região temporal esquerda, correspondente ao ACV na fase subaguda

Patología calcificante de las glándulas salivales: presentación de dos casos clínicos / Pathological calcification of the salivary glands: presentation of two clinical cases

La calcificación extraesquelética es una entidad patológica de depósito de sales minerales fuera de las estructuras óseas. Los casos frecuentes se presentan por obstrucción mecánica de la glándula salival o de su conducto excretor, asociada con la formación de sialolitos en el parénquima, su verdadera causa no se conoce con exactitud, pero hay distintas hipótesis con respecto a factores locales y sistémicos. La sialolitiasis es la afección más común de las glándulas salivales en adultos jóvenes, 12 de cada 1,000 pacientes sufren de esta patología, con un predominio del sexo masculino. El diagnóstico se puede establecer mediante ecografía, radiografías oclusales, panorámicas o tomografía cone beam. Estudiamos dos casos de calcificaciones de los tejidos glandulares. El primero de una formación mineral dentro de su conducto y el segundo de una calcificación completa del conducto salival (AU)

Extra bone calcification is a pathological entity of deposit of mineral salts outside bone structures. Frequent cases are due to mechanical obstruction of the salivary gland or its excretory duct, associated with the formation of sialoliths in the parenchyma, its true cause is unknown, but there are different hypotheses regarding the influence local and systemic factors. Sialolithiasis is the most common condition of the salivary glands in young adults, 12 out of 1,000 patients suffer from this pathology, with a predominance of males. Diagnosis can be established by ultrasound, occlusal radiographs, panoramic or cone beam tomography. We studied two cases of calcification of glandular tissues. The first is a mineral formation inside the duct and the second is a complete calcification of the salivary duct (AU)

Extraosseous Ewing's Sarcoma Presented as a Rectal Subepithelial Tumor: Radiological and Pathological Features

PURPOSE: Extraosseous Ewing's sarcoma (EOE) of the rectum is extremely rare: only three cases have been reported in the literature and none of these reports described their imaging findings in detail. Herein, we describe the tumor imaging and pathological features in detail. MATERIALS AND METHODS: We report a case of rectal EOE in a 72-year-old female who received local excision and was provisionally diagnosed with a rectal submucosal spindle cell tumor. We used immunohistochemistry, histopathology, and fluorescence in situ hybridization to characterize the tumor and provide a definitive diagnosis of EOE. RESULTS: MRI revealed a well-demarcated submucosal tumor with heterogeneous enhancement and hemorrhagic foci in rectum. EOE was diagnosed by positive staining of tumor cells for CD99 and Fli-1 by immunohistochemistry and the presence of the EWSR1 gene translocation by fluorescence in situ hybridization. Although the patient underwent radiation treatment and surgery, the tumor recurred after 4 months as revealed by computed tomography and magnetic resonance imaging. CONCLUSION: Rectal EOE may present as a rectal submucosal tumor. The understanding of imaging and histological characteristics of this tumor are critical for accurate diagnosis and appropriate aggressive treatment.

Sarcoma de Ewing extraóseo en espacio epidural / Extraosseous Ewing's sarcoma in spinal epidural space

El sarcoma extraóseo de Ewing pertenece a la familia de tumores neuroectodérmicos que derivan de la cresta neural, característico por su agresividad. Varias localizaciones se han descrito, sin embargo, la presentación epidural es rara. El pronóstico ha mejorado por nuevos esquemas oncológicos y están en estudio otras dianas terapéuticas.

Extraosseous Ewing's sarcoma belongs to the family of neuroectodermal tumors that derive from the neural crest, characteristic of its aggressiveness. Several locations have been described, however,epidural presentation is rare. Prognosis has been improved by new oncological regimens and other therapeutic targets are being studied.

Recurrent Peripheral Odontogenic Fibroma: A Case Report with the Review of Literature

Peripheral odontogenic fibroma (POF) is a rare benign mesenchymal odontogenic neoplasm with or without odontogenic epithelium, which is considered to be the mucosal analogue of central odontogenic fibroma. Though peripheral odontogenic tumours are rare, comprising of only 0.05% of all biopsy specimens, POF is the most common peripheral odontogenic tumour, more common than its central counterpart by a ratio of 1.4: 1. In this report, we describe a healthy 40-year-old female who was seen with a recurrent POF of the left mandible.

Peripheral Ameloblastoma of Mandible: A Rare Case Report.

Peripheral ameloblastoma (PA) is a rare, benign, extraosseous neoplasm of the odontogenic epithelium. It is an exophytic growth which is localized to the soft tissues overlying the tooth-bearing areas of maxilla and mandible. In most cases, there is no radiological evidence of bone involvement, except for cupping or saucerization of the underlying bone. Histologically, the PA consists of proliferating odontogenic epithelium that exhibits the same histomorphological cell types and patterns as seen in the intraosseous ameloblastoma. The PA is a slow growing and non-invasive, and recurrence is uncommon following excision, in contrast to central ameloblastoma which is locally invasive and can destroy large segments of the jaw. We report here a case of the plexiform type of PA.

Thoracic Extraosseous Epidural Cavernous Hemangioma

Cavernous hemangiomas were first reported in 1929 by Globus and Doshay, and are defined as benign vascular structures developed between the neural tissues occurring in the central nervous system, consisting of a dilated vascular bed. Cavernous hemangiomas comprise nearly 5-12% of all spinal vascular malformations; however, existence in the epidural space without bone involvement is rare. Only 4% of all cavernous hemangiomas (0.22/1.000.000) are purely epidural cavernous hemangiomas. In this case report, we removed a hemorrhagic thoracic mass presenting with progressive neurological deficits in a 55-year-old male patient. We found this case to be appropriate for presentation due to the rare occurrence of this type of cavernous hemangioma.

Paraspinal Extraosseous Ewing's Sarcoma With Disseminated Metastases Masquerading As Pott's Spine.

A 16 years old girl presented with low back pain, fever and weakness in both lower limbs for 3 months. Plain radiograph shows lytic lesion in first sacral vertebra and multiple lung nodules. In suspicion of tuberculosis, antitubercular drugs were advised. But magnetic resonance imaging revealed right paraspinal soft tissue mass with multiple lesions in several vertebrae causing spinal canal compression alongwith lesions in iliac bones, sacrum, lung parenchyma and scalp tissue. Fine needle aspiration cytology and biopsy revealed Ewing’s sarcoma. Patient was referred to higher centre for chemotherapy/radiotherapy. Herein we report this rare case of extraosseous Ewing’s sarcoma with disseminated metastases masquerading as Pott’s spine.

Ewing's Sarcoma of the Lesser Sac Masquerading as a Pancreatic Tumor

Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an uncommon, aggressive, and malignant tumor with a poor patient outcome. Its occurrence in the lesser sac is a rare event and to the best of our knowledge, has not been previously described. The present case was clinically and radiologically misdiagnosed as a pancreatic tumor/gastrointestinal stromal tumor. Histopathology revealed a tumor with "small round cells" that were positive for CD99, confirming the diagnosis of ES/PNET. This report highlights the importance of considering Ewing's sarcoma in the differential diagnosis of intraabdominal, extraintestinal masses.

Epithelial-mesenchymal transition in osteogenic sarcoma of the neck following oral squamous cell carcinoma

Postirradiation extraosseous osteogenic sarcomas are uncommon in the head and neck, despite the extensive use of high-dose radiation. It has been described as de novo radiation-induced neoplasm. We present a 73-year-old male who had been treated by radiotherapy for gingival cancer 7 years earlier and later developed extraosseous osteogenic sarcomas (EOSs) of the neck. Microscopically, the neck mass was composed with mesenchymal malignant cells with cartilaginous and osteogenic differentiation. Immunohistochemical stain demonstrated strong positivity of tumor cells for Snail, the one of major epithelial-mesenchymal transition (EMT) inducer. The E-cadherin expression was scarce, showing inverse relationship to Snail expression. Compared with previous squamous cell carcinoma (SCC) of the gingiva, the present EOS sample revealed the remained epithelial cells on cytokeratin immunohistochemistry, suggesting the tumor arise from the cells of epithelial origin. We have also reviewed the previous 6 cases of head and neck EOSs carefully. The clinicopathologic features of the unusual lesion suggest that it is an incomplete EMT of precedent epithelial malignancy rather than de novo pathology.

Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit.

Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.

Gingival peripheral odontoma in a child: case report of an uncommon lesion

Odontoma in an extraosseous location represents a challenge for diagnosis. This article reports a case of peripheral odontoma and its clinical presentation, histological evaluation and treatment. A 12-year-old boy reported a firm asymptomatic gingival mass in the anterior maxilla with two years of evolution. The procedures for diagnosis included intraoral examination, excisional biopsy and histological analysis. The diagnosis was peripheral odontoma. The follow-up revealed no sign of recurrence. Peripheral odontoma is rare and the differential diagnosis with other gingival masses is rather difficult and must include inflammatory and reactive processes. The definitive diagnosis is based on microscopic features.

Osteogenic Sarcoma with Osseous, Pulmonary, and Pericardial Metastases Simultaneously Demonstrated on Bone Scintigraphy at Initial Presentation / 대한핵의학회잡지

PURPOSE: A 6-year-old boy with osteogenic sarcoma of the left humerus underwent bone scintigraphy. Tc-99m MDP was accumulated not only in the primary tumor but also in the osseous and extraosseous (pulmonary and pericardial) metastases. Osteogenic sarcoma directly produces osteoid, both in the primary and metastatic lesions. Tc-99m MDP is avidly taken up by tumor osteoid. At initial presentation, only 2% of cases have both pulmonary and osseous metastases. The patient had osseous, pulmonary, and pericardial metastases at presentation. This case presents that increased uptakes of Tc-99m MDP by the primary and metastatic tumor were demonstrated on bone scintigraphy at presentation.

A Case of Extradural Spinal Tuberculoma

Extraosseous extradural tuberculosis of the spine is rare entity. A 21-year-old man with low back pain, left-sided sciatica & motor weakness was admitted. Simple X-ray were normal. At operation, extradural, extraosseous granuation tissue were removed, which proved upon histologic examination to be tuberculous granulomas. The authors report a rare case of extraosseous tuberculoma in the posterior extradural space of whole lumbar region with review of literatures.

A Case of Intracranial Meningeal Mesenchymal Chondrosarcoma: A Case Report

Recently, the authors have experienced a case of intracranial meningeal mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma is a rare tumor of the bone and soft tissue. It has been reported that the most common site of their extra-osseous origin is the central nervous system. Precise differential diagnosis should be done because of 1) its similarity to angioblastic meningioma or hemangiopericytoma in pathological aspect, 2) much more malignant tendency. We present one case of intracranial meningeal mesechymal chondrosarcoma with a brief review of the relevant literature.

A Case Report of Extraosseous Osteogenic Sarcoma / 대한정형외과학회잡지

Ex.traosseous osteogenic sarcoma is exceedingly rare although bone formation occurs commonly in different reactive processes or neoplasms. Most of this tumors occurred in the lower extremities of middle aged people. The overall prognosis is grave and local recurrence following simple local excision possibly contributes to the poor prognosis. An agressive surgical approach including major amputation is recommended as the primary form of treatment. We have experienced a case of extraosseous osteogenic sarcoma of left gluteal region at the site of therapeutic irradiation for cervical carcinoma 16 years previously.