Diagnosis by Image Coccygodynia due to an Immunoblastic Lymphoma
Genoveva Ochoa MD1, Alfonso Valenzuela-Espinoza MD2, John Domínguez3, Carlos SegoviaGarcía MD4, Víctor Whizar-Lugo MD5
Oncologist Clínica 20 Instituto Mexicano del Seguro Social Tijuana B.C., México 2 Patologist Hospital General de Tijuana ISESALUD Tijuana B.C., México 3 Medical Student Facultad de Medicina Universidad Autónoma de Baja California Tijuana B.C, México 4 Radilogist. Imagen Radiológica Integral. Tijuana B.C, México 5 Corresponsal author. Servicios Profesionales de Anestesiología y Medicina del Dolor Centro Médico del Noroeste Tijuana B.C., México
[email protected] 1
Abstract This article presents the case of a 63-year-old man who presented with severe, intermittent, dull, sacrococcygeal pain. The pain was worse in the seated position, upon waking, after long periods of standing, with lumbar hyperextension and during defecating. A lateral single x-ray was the key to suspecting a malignant lesion, which was confirmed by magnetic resonance image of the sacrum and coccyx. Excisional biopsy revealed an immunoblastic lymphoma, a rare type of non-Hodgkin’s lymphoma. Once the diagnosis was confirmed, the patient received CHOP-R and local radiotherapy. Good pain relief was obtained with high dose methadone, ketoprofen and maprotiline. To our knowledge this is the first case of an immunoblastic lymphoma manifesting as coccygodynia. Due to the rarity of sacral intraosseous involvement of this non-Hodgkin’s lymphoma, it is convenient to perform a full evaluation of those patients with severe coccygodynia and sacrococcygeal lesions. MRI images and a proper biopsy are essential to confirm the diagnosis. Key words: Coccygodynia, immunoblastic lymphoma.
Introduction
C
occygodynia, or coxalgia, is a relatively common well-known pain syndrome with a highly variable, not well understood etiology in which cancer is not usually a participating factor. Symptoms include sacrococcygeal pain, pelvic floor muscle spasms and referred pain secondary to
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lumbar pathology. The majority of cases of coccygeal pain are associated with abnormal movement of the coccyx, which results in chronic inflammation that may even cause degeneration of this anatomic region. Cases have also been associated with sacral root arachnoiditis, post traumatic lesions, metritis with retroversion, pathology of adnexa 52
and somatization disorder. Non-surgical management is preferred: cushions, massage, stretching, limiting the amount of time spent in the seated position, chiropractic manipulation with correction of poor posture, common analgesics, administration of local anesthetics with or without steroid deposit. Only a small percentage of patients with resistant pain require coccygeal resection, especially those with coccygeal subluxation or hypermobility, post traumatic coccygodynia and some neoplasias.1,2 In patients with severe incapacitating coccygodynia that impedes activities of daily life the physician must meticulously search for osteoarticular, muscular, neurological or neoplastic pathology in order to establish a definitive diagnosis and devise an appropriate therapeutic plan.3 A variety of primary and secondary neoplasms exist which have been described as causing coccygeal pain, with chordomas being the most frequent. Primary immunoblastic lymphoma of bone is a very rare subtype of non-Hodgkin’s lymphoma. It is characterized by its aggressive behavior and poor prognosis. The tumor contains immunoblasts with uniformly round or oval nuclei with one or more nucleoli and abundant cytoplasm. This morphology requires the establishment of a differential diagnosis with diffuse large B-cell lymphoma, with its centroblastic, anaplastic, plasma cell and clear cell variants, as well as angioimmunoblastic lymphoma of both B and T types, with poorly differentiated myeloma, which requires immunohistochemical study for correct morphologic and immunophenotypic identification. This rare tumor may be subdivided into plasma cell, clear cell or a third category named pleomorphic, which may appear similar to peripheral T-cell lymphomas.4,5 A differential diagnosis should be established with multiple myeloma. We have not encountered other reports of non-Hodgkin’s lymphoma associated with coccygodynia, and therefore report on an elderly patient with severe pain in the coccygeal region of neoplastic etiology, a diagnosis which was not considered at the beginning of evaluation and treatment.
Figure 1. Left panel is a lateral radiograph in which a lytic lesion of the coccyx is barely appreciable. This was confirmed through MRI, as seen in the center and right panels.
probing revealed a loss of 8 kg (17.6 lbs) in the previous month as well as occasional fever. There was no evidence of adenomegaly. In a lateral radiograph of the sacrococcygeal region we noted a lytic lesion suspicious of malignancy (left image of Figure 1). An MRI was performed, in which the lesion was confirmed (center and right images of Figure 1). An open biopsy of the lesion confirmed our suspicion of neoplasia, reporting a diffuse large B-cell lymphoma, CD-20 positive (Figure 2). Induction chemotherapy was initiated with CHOPRituximab, with maintenance therapy of rituximab. Response was poor, with evidence of primary resistance, and rescue attempts were made with DHEP. Pain was managed from the first consult with oral dexketoprofen 50 mg every 8 hours and tramadol 50 mg every 6 hours. Initial analgesic response was normal, and doses titrated accordingly: tramadol 100 mg every 6 hours, later substituted with methadone 30 mg every 6 hours, subsequently increased to 45 mg every 6 hours, then adding maprotiline 75 mg at night. This regimen finally decreased the pain to 3-4/10. The patient reported incidental pain crises upon sitting and defecating, which were managed with anticipatory administration of morphine, with partial response. The patient also received radiation therapy of the sacrococcyx as part of analgesic plan.
Description of the patient Patient is a 63 year old male with no important history, though he was a practicing anesthesiologist for 30 years, with exposure to anesthetic gas wastes, including nitrous oxide. He began with mild pain in the coccyx, appearing occasionally upon sitting. Over the course of three months the pain progressed from mild to severe, with a VAS of 9-10/10. The pain was intense, dull, did not radiate, prevented him from sitting, and increased on walking, on lumbar hyperextension and upon defecating. An orthopedic consult resulted in management as a simple coccygodynia with non-opiate analgesics PRN without satisfactory analgesic response. A lateral radiograph of the sacrum was interpreted as normal. The patient continued to experience severe pain, for which he self-prescribed dextropropoxyphene 65 mg, again without satisfactory analgesia. He was referred to our office with a request from the orthopedist that we apply an epidural steroid injection. The clinical history revealed no further data, and a physical exam reproduced the coccygeal pain upon applying pressure above the sacrococcygeal joint. Further
Figure 2. Diffuse large B-cell lymphoma, CD-20 positive. Proliferation of cells with large nuclei and one or two apparent nucleoli is identified. Note the abundant acidophilic cytoplasm and eccentric nucleus consistent with malignant lymphocytes with immunoblastic appearance. © Federación Mexicana de Anestesiología, A.C.
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Discussion Coccygodynia is not a common pathology in pain clinics as it tends to be adequately managed by orthopedists and/ or physical therapists. Sacrococcygeal pain may originate from the sacrococcygeal joints, contiguous structures with the same innervation or may be referred pain from another site, which is called pseudococcygodynia. The differential diagnosis may be established via meticulous physical examination, specialized image studies and in difficult cases it is possible to apply diagnostic nerve block using local anesthetics.6 One Japanese study7 evaluated 1000 healthy subjects and 500 patients with coccygeal pain and found that coccygodynia was more frequent in those patients in the fourth decade of life, as well as in women. The etiopathologic diagnosis of coccygodynia tends to be easy, as most cases involve abnormal movement of the coccyx, easily corroborated through the use of dynamic radiography.8 On occasion it is necessary to request radiographic study of intervertebral discs, bone gammagram, CT or MRI to establish the correct diagnosis. It is also occasionally necessary to request a biopsy. A small group of patients with coccygodynia may present with occult or undiagnosed tumor, as in this case report, where the patient went three months without a definitive etiologic diagnosis to explain his pain. The most frequent tumors which cause coccygeal pain are chrodomas.10 Other neoplasms which may be associated with this symptom are metastasis, teratomas, ependymomas of the filum terminale, precoccygeal glomus tumors, paragangliomas and benign tumors such as notochordomas and cysts.11-16 Lack of response to initial analgesic management and suspicion of a lytic lesion in simple X-ray lead us to suspect a tumor, which lead to ordering an MRI. This, together with a bone gammagram, are the most appropriate studies indicated in the second phase with these patients.8,17 The unexpected results of the biopsy gave us the key for oncologic management, as well as being necessary for immunohistochemical identification for differentiation between immunoblastic lymphoma vs. anaplastic myeloma. In the referenced literature we did not find cases of sacrococcygeal immunoblastic lymphomas as causes of coccygodynia. There are other non-Hodgkin’s lymphomas similar to immunoblastic lymphoma that are interesting to consider, with the idea of establishing the correct differential diagnosis. T-cells angioimmunoblastic lymphomas are another group of non-Hodgkin’s lymphoma that must be differentiated from immunoblastomas, as they affect lymph nodes and cause systemic manifestations such as high fever, rash, articular manifestations suggestive of arthritis, enteropathy, glomerulonephritis, pulmonary damage, tonsilar affectation, nasopharyngeal problems, etc.18-22 Multiple myeloma may also confound the diagnosis, though it principally affects bone tissue and is not as aggressive as immunoblastomas. Though steroids have been advocated as parte of the management of coccygodynea,23 they should not be administered prior to arriving at the correct definitive 54
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diagnosis. In the patient we present, premature injection of steroids without having a correct etiologic diagnosis, would have delayed even further the diagnosis of cancer and the analgesic effects would probably have been insufficient. At the same time, coccygeal resection should be reserved for extreme cases, as complications may be extreme. Chandawarkar24 reported on 50 patients with sacrococcygeal chordomas who underwent partial sacrococcygectomy, of which 14% suffered urinary incontinence, 6% rectal incontinence, 4% suffered hemorrhage and 2% rectal injury. The analgesic management of coccygodynia secondary to neoplasia is variable. Conservative management is preferred, as in our patient, searching for a combination of non-opiate analgesics and medium to potent narcotics, titrating the dose of each until a satisfactory result is obtained. Spaziante, et. al.,25 reported a case of oncologic sacroccoxygeal pain managed with implantation of a pump to administer intrathecal morphine. This article illustrates the importance of an integrated, interdisciplinary evaluation in the diagnosis and treatment of sacrococcygeal pain of oncologic origin, wherein radiographic and MRI imaging are vital for correct diagnosis, and we report the rare case of coccygodynia secondary to diffuse large B-cell lymphoma.
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