Journal of Cancer Research and Therapeutics Medknow Publications on behalf of the Association of Radiation Oncologists of India (AROI) ISSN: 0973-1482 EISSN: 1998-4138 Vol. 7, Num. 1, 2011, pp. 72-74

Journal of Cancer Research and Therapeutics, Vol. 7, No. 1, January-March, 2011, pp. 72-74

Case Report

Hypercalcemia with extraosseous MDP uptake in a bone scan as initial presentation in a case of cutaneous T-cell lymphoma

Shanmuga Sundaram Palaniswamy, S Padma, Vijay Harish, Jay Kumar Rai

Department of Nuclear Medicine and PETCT, Amrita Institute of Medical Sciences, Elamakkara Post, Cochin, Kerala, India

Correspondence Address: Shanmuga Sundaram Palaniswamy, Department of Nuclear Medicine and PETCT, Amrita Institute of Medical Sciences, Elamakkara Post, Cochin 682 041, Kerala, India, ssundaram@aims.amrita.edu

Code Number: cr11015

PMID: 21546747

DOI: 10.4103/0973-1482.80474

Abstract

Keywords: Bone scan, cutaneous T-cell lymphoma, extraosseous MDP uptake, mycosis fungoides

Introduction

Cutaneous T-cell lymphoma (CTCL) is a type of non-Hodgkin′s lymphoma. Unlike most non-Hodgkin′s lymphomas (which are generally B-cell related), CTCL is caused by a mutation of T-cell. The malignant T-cells produce cutaneous lesions to appear. These lesions change the shape as the disease progresses, typically beginning as what appears to be a rash to eventually forming plaques and tumors before metastasizing to other parts of the body.

Case Report

A 37-year-old lady presented with headache and vomiting of sudden-onset with intermittent fever, evening rise in nature. She also had slurring of speech with no loss of consciousness or altered sensorium. On close interrogation patient gave a history of hypopigmented dermal patches for more than 2 years duration. There was no history of muscle weakness, seizures, or lymphadenopathy.

On examination there were hypopigmented lesions with erythematous borders on the back. The patient also had bilateral facial palsy with higher functions being normal. Cranial nerves examination showed wrinkling of forehead to be decreased (Bells′ sign positive). Peripheral blood smear showed RBCs to be normocytic normochromic to hypochromic and mild anisocytosis. There is leukocytosis with scattered atypical lymphocytes, increased neutrophil/lymphocyte ratio and cribriform nuclei (20-25%). Platelets were markedly decreased. The patient had hypercalcemia (S. Calcium: 17.3 mg/dl) S. Creatinine was mildly raised (1.6 mg/dl) while Urine Bence Jones protein evaluation was negative. Her serum alkaline phosphatase level was high (158 IU/ml) and parathormone was found to be 10.56 pg/ml. Liver function tests were normal. Chest X-ray was normal.

The patient was thoroughly evaluated for pyrexia with investigations such as Widal, leptospiral antibody, human immunodeficiency virus, Hepatitis C Virus which were negative. Her CSF examination was normal. USG and CT abdomen showed the presence of medical renal disease bilaterally.

CT brain with contrast [Figure - 1] was reported as normal with sclerosis and thickening of bone. A facial nerve conduction study showed bilateral absent blink response raising a suspicion of brain stem lesion. At this juncture, a brain MRI was also performed which showed no brain stem lesion [Figure - 2]. As part of hypercalcemia evaluation, patient underwent a 99m Technetium Methylene Di Phosphonate (MDP) whole body bone scan and the scan showed intense uptake in facial and skull bones with extraosseous uptake of MDP in lungs, stomach and kidneys [Figure - 3]. Suspicion of CTCL was high in the background of hypercalcemia, extraosseous cause of MDP uptake in cutaneous lymphoma, and the presence of dermal patches. Skin biopsy were carried out which confirmed CTCL [Figure - 4] and [Figure - 5]. Bone marrow biopsy showed normocellular reactive bone marrow with no lymphomatous infiltration. The patient was started on Antibiotics, Tab. Acyclovir, Inj. Calcitonin, Inj. Pamidronate, and Wysolone. There was improvement in renal function, facial weakness, and in calcium level. Later she was planned for six cycles of chemotherapy.

Discussion

CTCL is also known as mycosis fungoidis, a classical form. It is an indolent variety of non-Hodgkin′s lymphoma with a male preponderance. ^[1] Children are rarely affected. Here the skin is infiltrated by lymphocytes. Its cause is unknown but in some patients it is associated with a pre-existing allergic dermatitis or retrovirus infection. It has a low-grade clinical course, which can persist in one stage for years or sometimes even decades. Progression from patches to thicker plaques occurs and eventually to produce tumors. ^[1]

In the patch stage of mycosis fungoides, the skin lesions are flat. Most often there are oval or ring-shaped (annular) pink dry patches on covered skin. They may spontaneously disappear, remain the same size, or slowly enlarge. The skin may be atrophic with or without any urticaria. The tumor stage of the disease causes lymphoma with or without involvement of supra and infradiaphragmatic lymph nodes and solid organs. With the availability of state of the art PET CT systems nowadays (Positron Emission Tomography integrated with conventional diagnostic Computed Tomography) staging and treatment response is best achieved.

T-cell lymphomas are associated with a higher incidence of hypercalcemia. ^[2] Hypercalcemia can be the first sign of B-cell lymphoma especially in HIV patients. ^[3]

The major causes of hypercalcemia in malignant lymphomas are increased bone resorption with associated renal disturbances. There can be an increase in the renal tubular calcium level. Hypercalcemia occurs frequently in myeloma and occasionally in patients with T-cell lymphomas, Hodgkin′s disease, or the B-cell lymphomas. ^[4] Of the T-cell lymphomas, only HTLV-I-associated lymphomas are frequently seen with hypercalcemia.

A 99m Technetium MDP bone scan in a patient with metabolic bone disease is typically described as a "beautiful bone scan". This is is due to the avid tracer uptake in the axial and appendicular skeleton with negligible soft tissue uptake. Kidneys may be nonvisualized or poorly visualized in these patients which is classically described as a "Superscan appearance". This is due to nonavailability of tracer to get cleared from kidneys as most of the tracer is strongly tagged to osteoblast by a chemisorption mechanism. However, in this case, we find striking extraosseous tracer accumulation in bilateral lungs, stomach, and kidneys indicating abnormal calcium deposition. Other classical findings usually seen in metabolic bone disease such as "Black beard sign" (abnormal increased mandibular tracer uptake), "Tie sternum sign" (linear increased tracer uptake in sternum), "Rosary beads sign" (Focal hot spots in bilateral costochondral junctions), superscan appearance, etc. were not present in this case.

The final diagnosis made in our patient was mycosis fungoides (CTCL), hypercalcemia with no hyperparathyroidism, and bifacial weakness (LMN palsy). While HTLV-1 is implicated as the cause of mycosis fungoides in our case, the associated facial weakness was assumed to be either due to Guillaine Barre Syndrome or to meningeal lymphoma. Hypercalcemia described with mycosis fungoides is usually one of primary hyperparathyroidism with raised PTH predisposing for cutaneous malignancy. ^[5]

References

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