A 43 year old male presented with complaints of left alveolar and left cervical swelling for past 5 month. We present summary of his case, minus the final diagnosis, to Professor XYZ, professor at KEMH, and asked him/her to reach a diagnosis

Left alveolar and left cervical swelling of 5 month duration

A 43-year-old male presented to the OPDs of Head and Neck services in November 2005. His chief complaints were left alveolar and left cervical swelling of 5 month duration.

Left alveolar swelling developed after painful tooth (infection) for which he saw a dentist who did extraction and gave him antibiotics (in July 2005). Subsequently he developed left cervical swelling, rapidly increasing in size. There was no history of fever, cough, expectoration, breathlessness, dyspnoea or weight loss. X-Ray chest was unremarkable.

He was diagnosis to have AIDS in 2000 when he consulted a general physician (in the railway hospital) for Herpes Zoster infection. His absolute CD4 count was 453cells/cmm at the time of diagnosis. He was started on AZT and had been on and off AZT in the past 4 years. His recent CD4+ T-lymphocyte count was 98cells/cmm. HIV I RNA quantitation for viral load was done by real time PCR was 287 copies/ml. The patient denied bowel or bladder incontinence, fever, chills, night sweats, weight loss, or any other systemic symptoms. No history of any surgical intervention in the past.

He was empirically put on anti tubercular treatment from August 2004 to Feb 2005 (details not available).

Both parents, wife and son are alive and well. No other family member is known to be seropositive. Maternal aunt had ovarian cancer.

Low socioeconomic status, he is working as a peon in the railways, with a monthly income of Rs. 3000. He is married and has one son who is 5 years old and healthy. 

He is a chronic tobacco chewer (gutka) for more than three decades. He occasionally drinks alcoholic beverages socially and smokes a packet of bidi per day for past two decades. There is no history of intravenous drugs abuse.

His risk factors for HIV were multiple heterosexual partners (commercial sex workers). He had no history of opportunistic infections and had agreed to initiate HIV therapy 4 months after the diagnosis.

He was empirically put on anti tubercular treatment from August 2004 upto Feb 2005. There was no relief in his symptoms. There was no history of any blood transfusion. Has been off and on AZT therapy.

On general physical examination, he appeared well (performance score - 2). He had mild pallor, no icterus, cyanosis or clubbing. He was afebrile. He did not have any palpable lymphadenopathy. Thyroid gland was normal.

Weight: 54 kg, Height: 5 feet 4 inches, Temperature: 97.5, Blood pressure 126/82 mm Hg, Pulse 66/minute.

Cardiovascular examination: Heart rate and the rhythm were normal. No murmur or any carotid bruits.

Neurological examination was normal.

Lungs: Clear to auscultation bilaterally. No crepts or rhonchi.

Abdomen: Non distended soft abdomen. No palpable mass. Normoactive bowel sounds. No rebound tenderness. No inguinal hernias or inguinal lymphadenopathy.

Genitalia: Pens, bilateral testicles were normal.

Extremities: No pedal edema.

1. Clinical appearance of the patient showed an asymmetric face owing to the big left alveolar swelling. It was accompanied with left sided proptosis and bulging of the hard palate. There was oozing from the nasal cavity. There was an ulceroproliferative left alveolar swelling, 3x3 cm, extending to left palatal and the alveolar ridge. The swelling was covered by a yellowish membrane.

2. Left cervical swelling, 15x20 cms in size, non tender, non mobile, fixed to the underlying soft tissues.

3. Multiple small subcutaneous swellings (cervical region, anterior abdominal wall, right shoulder), largest 2x1 cm in size. These nodules are of similar duration.

An incisional biopsy was performed from the left alveolar lesion, and the specimen fixed in 10% neutral formalin solution

1. Radiology investigations:

a) X-Ray chest was within normal limits.

b) Ultrasound abdomen revealed splenomegaly, small peripancreatic nodes.

c) Whole body PET-CT was done (figures attached)

2D-Echocardiography revealed normal left ventricular systolic function. The left ventricular ejection fraction was 65%.

2. Laboratory investigations: At presentation to our hospital, his complete blood counts were within normal ranges. WBC 5900/cmm; Hemoglobin 11.5gm/dL; Platelet 205,000/cmm. Coagulation profile: PT 10.1; APTT 27.9. Peripheral blood smear revelaed a normocytic normochromic picture, normal differential count (neutrophils 64%, lymphocytes 33% and monocytes 3%) and adequate platelet count. Liver function tests and renal function tests were within normal ranges.
Blood urea 19mg%, Serum creatinine 1.5mg%, serum uric acid 5.5mg%, Na 139 mmol/L; K 4.0 mmol/L; Cl 103 mmol/L, Glucose 94; Calcium 8.3; TP 5.9; Total Bilirubin 0.2; AST 29; ALT 44; Alk Phos 93
ESR was 32 mm Hg, Serum proteins were mildly raised to 9.3 gm% (6.4-8.3), Serum albumin was 3.4 gm% (3.5-5.2). Serum globulins were raised to 5.9 gm% (1.7-3.5) as was SGOT values of 82U/L (10-37). Serum electrophoresis revealed polyclonal hypergammaglobulinemia.
LDH 502IU/L; beta 2 microglobulin 8.71U/L

3. Microbiology investigations: Bacterial, fungal, and mycobacterial cultures were normal. Tuberculin skin test was non-reactive.

4. HIV testing done by ELISA:.HIV I RNA quantitation for viral load was done by real time PCR was 563.copies/ml

Lymphocyte enumeration, peripheral blood (by flow cytometry ) reveled:

Total leucocytes 6200/cmm
Lymphocytes 34.0%
Absolute lymphocyte count 1,768/cmm
CD3+/CD4+ percentage 7.61%
Absolute CD4 count 134/cmm
CD3+/CD8+ percentage 50.0%
Absolute CD8 count 884/cmm
CD4/CD8 ration 0.15
Hemoglobin blood 12.0 g/dL
Platelet cont 207 x 1000/cmm

5. Histopathology: Punch biopsy of left upper alveolus was done in Novemebr 2005. It showed a high grade malignant tumor with marked apoptotic and mitotic activity admixed with chronic inflammatory infiltrate. It revealed a monotonous diffuse proliferation of large sized tumor cells with moderately bluish agranular cytoplasm. There was no squamoid differentiation in form of keratin and no pigment could be demonstrated.
Immunohistochemistry (IHC) was done for further characterization of the tumor. Hematolymphoid markers (LCA, CD20, CD3, CD30, Alk-1), epithelial markers (CK, EMA), melanoma markers (S-100 protein, HMB-45), myogenic markers (Desmin, Myoglobin, Myogenin, MyoD1), and neurogenic markers (Mic2, NSE) were negative.

6. Cytology: FNAC from the cervical swelling revealed a hemorrhagic non-diagnostic aspirate.