# Cytological Perspective in a Case of Doege-Potter Syndrome With Hypoinsulinemic Hypoglycemia

**Authors:** Ingitha Pulikkal, Mahadev Meena, Garima Goel, Abhishek Goyal, Tanya Sharma

PMC · DOI: 10.7759/cureus.56041 · Cureus · 2024-03-12

## TL;DR

This paper describes a rare lung tumor case with hypoglycemia, highlighting its cytological features and diagnostic challenges.

## Contribution

The study presents a unique case of Doege-Potter syndrome with detailed cytomorphological analysis of a solitary fibrous tumor.

## Key findings

- The tumor exhibited low-grade mesenchymal features with specific immunohistochemical markers.
- Hypoglycemia was linked to the tumor, emphasizing the importance of early cytological diagnosis.
- NAB2-STAT6 fusion is a key molecular feature of the tumor.

## Abstract

Solitary fibrous tumor (SFT) of the lung is a rare mesenchymal neoplasm of uncertain histogenesis, unknown molecular features, and unpredictable clinical behavior, characterized by NAB2-STAT6 fusion. Hypoglycemia accompanying SFT (Doege-Potter syndrome) is an uncommon presentation. We present the cytomorphological features on biopsy imprint smears of a histopathologically confirmed case of SFT of the lung with an uncommon presentation. A 76-year-old non-smoker, non-alcoholic, and non-diabetic man presented with complaints of intermittent episodes of confusion with syncopal attacks (>10 episodes) for six months. The patient had no respiratory complaints and no history of weight loss. Laboratory investigations revealed fasting blood sugar of 38 mg/dl with low serum insulin and C-peptide levels. Physical examination revealed reduced air entry on the left side of the chest. Chest X-ray showed left-sided homogenous opacity. High-resolution computed tomography (HRCT) of the chest showed a large left-sided lung mass. A biopsy was performed. Biopsy imprint smears were cellular and showed tumor cells arranged in clusters and fragments with traversing capillaries displaying monomorphic pump to oval nuclei, fine granular evenly dispersed chromatin, regular nuclear membrane, inconspicuous nucleoli, and a moderate amount of wispy cytoplasm. Foci of intercellular hyaline stromal material were noted. A cytodiagnosis of low-grade mesenchymal neoplasm was made. Histopathology revealed a cellular tumor comprising tightly packed round to fusiform cells arranged around blood vessels with intervening thick collagen, positive for CD99, vimentin, BCL2, CD34, and STAT6 and negative for EMA, CK AE1/AE3, S100, TLE1, and SMA. Familiarity with cytomorphology plays a pivotal role in clinching an early diagnosis of this rare neoplasm of the lung, particularly in the setting of presentation with hypoglycemia.

## Linked entities

- **Proteins:** CD99 (CD99 molecule (Xg blood group)), PRELID1 (PRELI domain containing 1), BCL2 (BCL2 apoptosis regulator), CD34 (CD34 molecule), STAT6 (signal transducer and activator of transcription 6), ETFA (electron transfer flavoprotein subunit alpha), S100A1 (S100 calcium binding protein A1), TLE1 (TLE family member 1, transcriptional corepressor), SMN1 (survival of motor neuron 1, telomeric)
- **Diseases:** hypoglycemia (MONDO:0004946)

## Full-text entities

- **Genes:** CD34 (CD34 molecule) [NCBI Gene 947], BCL2 (BCL2 apoptosis regulator) [NCBI Gene 596] {aka Bcl-2, PPP1R50}, TLE1 (TLE family member 1, transcriptional corepressor) [NCBI Gene 7088] {aka ESG, ESG1, GRG1, TLE-1}, NAB2 (NGFI-A binding protein 2) [NCBI Gene 4665] {aka MADER}, MUC1 (mucin 1, cell surface associated) [NCBI Gene 4582] {aka ADMCKD, ADMCKD1, ADTKD2, CA 15-3, CD227, Ca15-3}, VIM (vimentin) [NCBI Gene 7431], CD99 (CD99 molecule (Xg blood group)) [NCBI Gene 4267] {aka HBA71, MIC2, MIC2X, MIC2Y, MSK5X}, S100A1 (S100 calcium binding protein A1) [NCBI Gene 6271] {aka S100, S100-alpha, S100A}, INS (insulin) [NCBI Gene 3630] {aka IDDM, IDDM1, IDDM2, ILPR, IRDN, MODY10}, STAT6 (signal transducer and activator of transcription 6) [NCBI Gene 6778] {aka D12S1644, HIES6, IL-4-STAT, STAT6B, STAT6C}, SMN1 (survival of motor neuron 1, telomeric) [NCBI Gene 6606] {aka BCD541, GEMIN1, SMA, SMA1, SMA2, SMA3}
- **Diseases:** neoplasm of the lung (MESH:D008175), SFT (MESH:D054364), mesenchymal neoplasm (MESH:D009369), confusion (MESH:D003221), diabetic (MESH:D003920), weight loss (MESH:D015431), lung mass (MESH:D008171), Hypoinsulinemic Hypoglycemia (OMIM:240900), Doege-Potter Syndrome (MESH:C536482), syncopal attacks (MESH:D013575), Hypoglycemia (MESH:D007003)
- **Chemicals:** blood sugar (MESH:D001786), C-peptide (MESH:D002096)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11007478/full.md

## References

20 references — full list in the complete paper: https://tomesphere.com/paper/PMC11007478/full.md

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Source: https://tomesphere.com/paper/PMC11007478