Dr Theresa, Dr Praveena,DrC Jayakumar (Amrita Institute of Medical sciences, Kochi)
1Twelve year old male child s/p TAPVC repair presented with complaints of a swelling noted in the right side of neck since 1 month duration. Initially the size was 0.5 X 0.5cm soft, non tender , mobile, with no erythema but increased in size in the last 2 weeks durationto 4 X 4 cm firm to hard in consistency, not mobile and non tender. He also had fever associated with night sweats of 3 days duration. History of cough of 2 weeks duration was also present . He also had a dental extraction 1 month prior to swelling. USG neck was done outside which showed coarse thyroid echotexture with lesion in right posterior triangle.
History is negative for evening rise of temperature, malaise, rash, joint pain, conjunctival congestion, dysphagia, hoarseness of voice, chest pain, dyspnoea, dysuria, abdominal pain, loose stools.
At 1 n half years of age, in view of breathing difficulty, ECHO was done which showed supracardiac TAPVC and repaired at 2 years of age.
The child is developmentally normal and immunized for age.
At admission, the child was febrile with other vitals stable. On examination right posterior cervical lymph node palpable- 4 X 4cm, non tender, firm to hard in consistency, non mobile and left posterior cervical lymph node palpable <0.5cm
No pallor, icterus, cyanosis, clubbing, oedema. Head to foot examination showed sternotomy scar
Auxology was normal for age.
Systemic examination showed congested tonsillar pillar.
Labs normal counts with negative CRP. RFT, LFT
Serum electrolytes and LDH done were normal.
Peripheral smears showed mild eosinophilia with thrombocytosis.
EBV IgM was equivocal and
Toxoplasma IgM was negative.
Quantiferon TB gold was also negative.
The child was started on Tab Cefadroxil 30mg/kg
USG neck showed heterogeneous appearing solid lesions in the right posterior triangle with no features of active inflammation – ? nodal mass. Thyroid gland showed features of thyroiditis.
Pediatricsurgery consultation was availed and cervical lymph node biopsy was done that reported benign spindle cell neoplasm, morphology featuring proliferative fasciitis. He is planned for excision now.
Discussion:
Benign spindle cell neoplasm:
Benign spindle cell neoplasm of the neck refers to a non – cancerous tumour made up of spindle shaped cells, which are elongated cells that resemble a spindle. The tumours can arise from various tissues in the neck, including smooth muscle, nerve sheath cells or fibroblasts. Common benign spindle cell neoplasm in the neck include:
1) Schwannoma : tumour arising from the nerve sheath cells
2) Leiomyoma: originating from smooth muscle cells.
3) Fibromatosis: Benign proliferation of fibroblasts
4) Solitary fibrous tumour: Type of soft tissue tumour that can occur in various areas of the body including the neck
General pathological features of benign spindle cell neoplasm
These tumous shows spindle shaped cell with elongated nuclei and cytoplasm. In benign cases, there is typically low mitotic activity, minimal cellular pleomorphism and no evidence of necrosis or significant atypia
Markers such as S-100, smooth muscle actin, CD34, helps in distinguishing the specific type of spindle cell neoplasm. The tumours are usually well circumscribed and slow growing ,with limited potential to invade surrounding structures or metastasize. However some may be locally aggressive like fibromatosis
While these lesions are non cancerous , their location in the neck can sometimes lead to symptoms like pain, a palpable mass or nerve compression. Diagnosis is typically made through imaging studies and confirmed with biopsy
Treatment is usually surgical excision with complete removal with clear margins to prevent recurrence.