Diagnose von Weichteiltumoren in der Zytologie

Zusammenfassung: Weichteilschwellungen sind das Symptom einer Vielzahl von neoplastischen und nichtneoplastischen Veränderungen. Sarkome gehören zu den selteneren Ursachen. Die Feinnadelpunktion hat sich in der Abklärung von Tumorrezidiven und Karzinommetastasen als minimal-invasive, kostengünstige und zuverlässige Methode bewährt. Auch in der Abklärung von Weichteiltumoren kann sie zu einer präzisen Diagnose führen. Voraussetzung ist eine enge multidisziplinäre Zusammenarbeit unter Einbeziehung klinischer, radiologischer und morphologischer Befunde. Für die zytologische Befundung sind Alter und Geschlecht sowie Topographie, Größe und Wachstumsgeschwindigkeit des Tumors wichtige Parameter. Reifungsgrad und Form der Zellen sowie Vorhandensein und Ausdifferenzierung der bindegewebigen Matrix bieten erste differenzialdiagnostische Hinweise und sind der Ausgangspunkt für immunzytochemische und molekularbiologische (FISH, RT-PCR) Zusatzuntersuchen. Diese Untersuchungen werden an Direktausstrichen, an mittels Zellblockmethode eingebettetem und an tiefgefrorenem Punktionsmaterial ausgeführ

Zytologische Sarkomdiagnostik

Zusammenfassung: Weichteilschwellungen sind das Symptom einer Vielzahl neoplastischer und nichtneoplastischer Veränderungen. Sarkome gehören zu den selteneren Ursachen. Die Feinnadelpunktion (FNP) hat sich in der Abgrenzung zu entzündlichen Prozessen sowie in der Abklärung von Tumorrezidiven und Karzinommetastasen als minimal-invasive, kostengünstige und zuverlässige Methode bewährt. Auch in der Abklärung von Weichteiltumoren kann sie zu einer präzisen Diagnose führen. Voraussetzung ist eine enge multidisziplinäre Zusammenarbeit unter Einbeziehung klinischer, radiologischer und morphologischer Befunde. Für die zytologische Befundung sind Alter und Geschlecht sowie Topographie, Größe und Wachstumsgeschwindigkeit des Tumors wichtige Parameter. Reifungsgrad und Form der Zellen sowie Vorhandensein und Ausdifferenzierung der bindegewebigen Matrix bieten erste differenzialdiagnostische Hinweise und sind der Ausgangspunkt für immunzytochemische und molekularbiologische (FISH, RT-PCR) Zusatzuntersuchungen. Diese Untersuchungen werden an Direktausstrichen, an mittels Zellblockmethode eingebettetem und an tiefgefrorenem Punktionsmaterial ausgeführ

Late solitary bone metastasis of a primary pulmonary synovial sarcoma with SYT-SSX1 translocation type: case report with a long follow-up

Primary synovial sarcoma outside its classical presentation in para-articular soft tissue of young patients is rare but regularly reported. One of the rarest primary locations is the lung. We describe a 73-year-old female patient who presented with a solitary malignant bone tumor 8years after the resection of a lung neoplasm. The bone tumor was classified as an osteosarcoma and the lung tumor as an atypical carcinoid tumor at their first respective diagnostic work-ups. The resection of the affected humerus with allograft and endoprosthesis implantation followed. Reevaluation of the tumor samples at the time of the local recurrence of the bone tumor 6years following the initial symptoms of the bone tumor lead to the reclassification of both specimens as synovial sarcomas. Both neoplasms contained the SYT-SSX1 type of the diagnostic translocation t(X;18) as detected by the reverse-transcription polymerase chain reaction analysis. The patient died 14years after the resection of the primary synovial sarcoma of the lung and 6years following the occurrence of the bone metastasis. This prolonged clinical course is uncommon for the SYT-SSX1 translocation, which, in other locations, is usually associated with an unfavorable prognosi

Response of pulmonary artery intimal sarcoma to surgery, radiotherapy and chemotherapy: a case report

<p>Abstract</p> <p>Introduction</p> <p>Pulmonary artery intimal sarcoma is a rare disease with no characteristic symptoms. It is difficult to diagnose early and is frequently misdiagnosed as a pulmonary embolism.</p> <p>Case presentation</p> <p>Here we report a case of pulmonary artery intimal sarcoma in a 54-year-old woman presenting with complaints of shortness of breath on exertion. Echocardiography and a computed tomography scan showed that the right pulmonary artery trunk was blocked by a low-density mass. The patient was diagnosed with pulmonary artery intimal sarcoma by pathology and a complete mass resection was performed. After experiencing 10 months of disease-free survival, she was re-admitted because of the recurrence and metastasis of the tumor. Radiotherapy and chemotherapy were performed; however, only limited success was achieved. The patient died 15 months after the initial onset of symptoms.</p> <p>Conclusion</p> <p>Some patients with intimal sarcoma of the pulmonary artery can benefit from radiotherapy and chemotherapy as well as surgery.</p

Intimal aortic sarcoma mimicking ruptured thoracoabdominal type IV aneurysm. a rare case report and review of the literature

Primary intimal aortic sarcoma represents a very rare and highly lethal medical entity. Diagnosis is made either by embolic events caused by the tumor or by surrounding tissue symptoms such as pain. Herein we report an extremely rare case of a 51-year-old man previously operated for ascending aortic aneurysm, who presented with clinical and radiological findings suggestive of a ruptured thoracoabdominal type IV aneurysm. The patient underwent radical resection of the aorta and surrounding tissue with placement of a composite 4-branched graft. The diagnosis was made by frozen section and regular histopathologic examination of the specimen and the patient received adjuvant chemotherapy. Nine months after surgery the patient is still alive and has no signs of recurrence. We review the literature and discuss the option of postoperative chemotherapy

Chronic recurrent Gorham-Stout syndrome with cutaneous involvement

Type IV osteolysis or Gorham-Stout syndrome is a rare condition characterized by recurrent vascular tumors that disrupt normal anatomical architecture. Gorham-Stout syndrome is most commonly associated with the skeletal system with resulting replacement of bone with scar tissue following tumor regression. The loss of entire bones has given Gorham-Stout syndrome the moniker vanishing bone disease. Natural progression of Gorham-Stout syndrome is characterized by spontaneous disease resolution. However, rare variants of recurrent, progressive, and/or systemic disease have been reported. We present a patient with a history of recurrent Gorham- Stout disease refractory to all treatment options considered. In addition to skeletal disease, our patient had soft tissue and cutaneous involvement, thus reflecting the more aggressive disease variant. Previous surgical attempts to control disease had been ineffective and the patient was referred to us for radiation therapy. Treatment with external beam radiation therapy resulted in good local control and symptom palliation, but full disease resolution was never accomplished. In addition to presentation of this patient, a review of the literature on etiological hypotheses and past/future treatment options was conducted and is included

The diagnosis and management of patients with idiopathic osteolysis

Idiopathic osteolysis or disappearing bone disease is a condition characterized by the spontaneous onset of rapid destruction and resorption of a single bone or multiple bones. Disappearing bone disorder is a disease of several diagnostic types. We are presenting three patients with osteolysis who have different underlying pathological features. Detailed phenotypic assessment, radiologic and CT scanning, and histological and genetic testing were the baseline diagnostic tools utilized for diagnosis of each osteolysis syndrome. The first patient was found to have Gorham-Stout syndrome (non-heritable). The complete destruction of pelvic bones associated with aggressive upward extension to adjacent bones (vertebral column and skull base) was notable and skeletal angiomatosis was detected. The second patient showed severe and aggressive non-hereditary multicentric osteolysis with bilateral destruction of the hip bones and the tarsal bones as well as a congenital unilateral solitary kidney and nephropathy. The third patient was phenotypically and genotypically compatible with Winchester syndrome resulting in multicentric osteolysis (autosomal recessive). Proven mutation of the (MMP2-Gen) was detected in this third patient that was associated with 3MCC deficiency (3-Methylcrontonyl CoA Carboxylase deficiency). The correct diagnoses in our 3 patients required the exclusion of malignant osteoclastic tumours, inflammatory disorders of bone, vascular disease, and neurogenic arthropathies using history, physical exam, and appropriate testing and imaging. This review demonstrates how to evaluate and treat these complex and difficult patients. Lastly, we described the various management procedures and treatments utilized for these patients

Cytological diagnostic features of late breast implant seromas. From reactive to anaplastic large cell lymphoma

Late breast implant seroma may be the presentation of a breast implant-associated anaplastic large cell lymphoma (BI-ALCL), which claims for a prompt recognition. However, BI-ALCL diagnosis on fine-needle aspiration (FNA) might be challenging for pathologists lacking experience with peri-implant breast effusions. Sixty-seven late breast implant seromas collected by FNA from 50 patients were evaluated by Papanicolaou smear stain and immunocytochemistry on cell blocks. A diagnostic algorithm based on the cellular composition, cell morphology and percentage of CD30+ cells was developed. Histological evaluation of the corresponding peri-prosthetic capsules was also performed. Most of the effusions (91% of the samples) were classified as reactive and 9% as BI-ALCL. In the BI-ALCL cases, medium-to-large atypical cells expressing CD30 represented more than 70% of the cellularity, whereas in in the reactive effusions CD30+ elements were extremely rare (<5%) and consisted of non-atypical elements. The reactive effusions were categorized into three patterns: i) acute infiltrate with prominent neutrophilic component (33% of the samples); ii) mixed infiltrate characterized by a variable number of neutrophils, lymphocytes and macrophages (30% of the samples); iii) chronic infiltrate composed predominantly of T lymphocytes or macrophages with only sporadic granulocytes (37% of the samples). The inflammatory cytological patterns were consistent with the histology of the corresponding capsules. Our results indicate that cytological analysis of late breast implant effusions, supported by the knowledge of the heterogeneous cytomorphological spectrum of late seromas, is a valuable approach for the early recognition of BI-ALCL

Diagnose von Weichteiltumoren in der Zytologie

Weichteilschwellungen sind das Symptom einer Vielzahl von neoplastischen und nichtneoplastischen Veränderungen. Sarkome gehören zu den selteneren Ursachen. Die Feinnadelpunktion hat sich in der Abklärung von Tumorrezidiven und Karzinommetastasen als minimal-invasive, kostengünstige und zuverlässige Methode bewährt. Auch in der Abklärung von Weichteiltumoren kann sie zu einer präzisen Diagnose führen. Voraussetzung ist eine enge multidisziplinäre Zusammenarbeit unter Einbeziehung klinischer, radiologischer und morphologischer Befunde. Für die zytologische Befundung sind Alter und Geschlecht sowie Topographie, Größe und Wachstumsgeschwindigkeit des Tumors wichtige Parameter. Reifungsgrad und Form der Zellen sowie Vorhandensein und Ausdifferenzierung der bindegewebigen Matrix bieten erste differenzialdiagnostische Hinweise und sind der Ausgangspunkt für immunzytochemische und molekularbiologische (FISH, RT-PCR) Zusatzuntersuchen. Diese Untersuchungen werden an Direktausstrichen, an mittels Zellblockmethode eingebettetem und an tiefgefrorenem Punktionsmaterial ausgeführ