Epithelioid angiosarcoma [Neurosurgery Wiki]

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====== Epithelioid angiosarcoma ======

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Epithelioid angiosarcoma is a rare, highly aggressive malignant tumor arising from vascular endothelial cells, characterized by epithelioid morphology—i.e., tumor cells that resemble epithelial cells in appearance but are of endothelial origin.

Key Features:

Histology: Large, pleomorphic epithelioid cells with abundant eosinophilic cytoplasm, prominent nucleoli, and frequent mitoses. Often displays areas of necrosis and hemorrhage.

Immunohistochemistry: Positive for CD31, ERG, FLI-1, and CD34; often negative for epithelial markers like cytokeratin (though focal positivity can occur, contributing to misdiagnosis).

Clinical Behavior: Extremely high rate of local recurrence and distant metastasis (lungs, liver, bone), with poor prognosis—median survival often less than 12 months.

Common Sites: Deep soft tissues (e.g., pelvis, limbs), skin, breast, pleura, bone, and rarely the spine or CNS.

Etiology: Sometimes associated with chronic lymphedema, prior radiation exposure, or foreign material (e.g., vascular grafts), though most cases are idiopathic.

===== Case reports =====
Nan et al.
((Nan YH, Chiu CD, Chen WL, Chen LC, Chen CC, Cho DY, Guo JH. [[Epithelioid angiosarcoma]] of the [[cervical spine]]: A [[case report]]. World J Clin Cases. 2025 Jun 16;13(17):101593. doi: 10.12998/wjcc.v13.i17.101593. PMID: 40524767; PMCID: PMC11866273.))
describe a rare case of epithelioid [[angiosarcoma]] (EA) involving the [[cervical spine]], presenting with [[pathological fracture]] and [[kyphotic deformity]], and document the surgical and [[adjuvant]] [[management]] as well as the [[clinical outcome]] in the [[World Journal of Clinical Cases]].

=== 1. Predictable Yet Pointless ===
The [[author]]s claim [[novelty]] by describing a rare anatomical presentation of EA. However, this degenerates into a predictable narrative with **no new pathophysiological insights, no hypothesis generated**, and **no clinical paradigm challenged**. It is the kind of “rare case” that proliferates in low-barrier journals precisely *because* it demands no [[intellectual risk]].

=== 2. Zero Diagnostic Value ===

The authors bypass the opportunity to deepen our understanding of the **radiological-morphological signature** of EA in the spine. No comparative imaging, no differential diagnostic flowchart, no histopathological discussion beyond standard [[CD31]]/[[CD34]] immunostaining. If this case had been published in 1995, it would be equally uninformative.

=== 3. Therapeutic Confusion Disguised as Aggressiveness ===

Two major spine surgeries (posterior [[decompression]] + anterior [[corpectomy]]) followed by **immediate [[radiotherapy]]** in a [[moribund]] patient demonstrate **[[therapeutic overreach]] without oncological [[strategy]]**. There is no discussion on multidisciplinary planning, palliative thresholds, or whether delaying surgery or avoiding the second procedure might have prevented ARDS. The reader is left with the impression of a **surgical reflex**, not an evidence-based decision.

=== 4. No Discussion of Differential Diagnosis or Biomarkers ===

In a tumor type notorious for being misdiagnosed as [[metastasis]], [[chordoma]], or [[sarcoma]] NOS, the absence of a **differential diagnostic framework** or advanced markers (ERG, FLI1, HHV-8, etc.) is alarming. **Histological laziness** cloaked in “rare disease” rhetoric.

=== 5. Outcome Reporting: Conveniently Truncated ===

The patient dies 3 weeks after surgery, yet the discussion **fails to draw any causal or cautionary link** between the interventions and the fatal ARDS. No [[autopsy]] data, no postmortem imaging, no pulmonary workup. This **omission sterilizes the clinical narrative**, reducing it to [[anecdote]].

=== 6. Ethically Murky ===

The case implicitly raises an [[ethical dilemma]]—should maximal surgery be performed in aggressive, terminal tumors without demonstrated systemic control? Yet the authors shy away from even mentioning this, let alone framing it for academic discussion.

=== 7. Journal Choice Reflects the Paper’s Weakness ===

Published in a journal known for **minimal peer review stringency**, the article offers **no [[citation]]s of recent molecular or [[targeted therapy]] advances**, no engagement with broader oncological guidelines, and no rationale for the treatment decisions beyond procedural listing.

===== Definitions (for Teaching Purposes) =====

  * **Histological laziness:** Failing to provide in-depth pathology discussion beyond CD31/CD34 and H&E staining in vascular tumors.
  * **Surgical reflex:** The tendency to operate based on mechanical findings (compression, fracture) without integrating prognosis or systemic disease behavior.
  * **Ethical sterilization:** Avoiding uncomfortable questions about futility, risk-benefit tradeoffs, and overtreatment in end-stage patients.
  * **Postmortem evasion:** Reporting a perioperative death without diagnostic closure (autopsy, imaging, or medical reflection).

===== Conclusion =====
This case report is **an example of procedural reporting devoid of scientific merit**, clinical reflection, or ethical introspection. It contributes **nothing to the understanding of EA**, its diagnosis, biology, or management—beyond reiterating its rarity. In its current form, it is **neither [[hypothesis-generating]] nor [[practice-changing]]**, and serves as a **cautionary tale on how not to write a [[case report]]**.

===== Suggested Revisions =====
  * Include comparative radiology with metastatic disease and primary bone tumors.
  * Provide autopsy findings or detailed explanation of respiratory decline.
  * Discuss therapeutic alternatives (e.g., single-stage surgery, biopsy + RT, palliative care).
  * Frame the case within an oncological decision-making algorithm.