Ashok K. Malani

Family history in necrotising fasciitis

In the Case Report on necrotising fasciitis of the breast by Rachel Tillett and colleagues (July 8, p 174), there were features on admission that we believe would point to the possibility of necrotising fasciitis. These were tachycardia, hypotension, erythematous swollen breast without evidence of abscess formation, pain dispropor tionate to local fi ndings, and worsening pain despite antibiotic coverage. Additionally, Tillett and colleagues mention that a history of gastroenteritis and family history of strepto coccal illness was overlooked initially, and that this was one of the clues to the diagnosis of necrotising fasciitis. However, a lack of such history should not have prevented clinical diagnosis. Predisposing factors include history of blunt trauma, varicella (chickenpox), injection drug use, penetrating injury such as laceration, surgical procedures, child birth, burns, and perhaps nonsteroidal anti-infl ammatory drugs. Although the presence of a family history of streptococcal-type illness in this case was interesting, too much reliance on this aspect of clinical history could be dangerous. Lastly, there is no strong published evidence to support penicillin prophylaxis to all family contacts: the patient’s baby would be the only one at a very high risk. In conclusion, a high index of suspicion and low threshold for surgical consultation leads to early diagnosis and appropriate treatment of this treatable but potentially fatal condition.

Metastatic retroperitoneal epithelioid angiomyolipoma

Angiomyolipoma (AML) is a rare benign neoplasm that usually arises in the kidneys, but may rarely originate in sites such as the retroperitoneum, liver and bone. It is characterised by an intimate admixture of blood vessels, smooth muscle and fat. This multiphasic composition allows for its recognition on ultrasound and CT scan examination. Metastases are exceedingly uncommon, and only one other case of metastatic extrarenal AML exists in the literature. Histological variants of AML such as epithelioid angiomyolipoma (EAML) are considered to be locally aggressive. A unique case of an EAML of the retroperitoneum that metastasised to the liver and bone in an 80-year-old woman is described.

A case of paraneoplastic cerebellar ataxia secondary to renal cell carcinoma

A 64-year-old female patient presented with acute cerebellar ataxia. After ruling out vascular, infectious, metastatic, demyelinating, and medication etiologies, a paraneoplastic origin for her illness was determined. A renal mass was discovered and found to be renal cell carcinoma. Her symptoms resolved after a radical nephrectomy. Physicians should consider the diagnosis of paraneoplastic neurologic syndromes whenever a thorough evaluation fails to explain neurologic abnormalities.

Concomitant presentation of acute myeloid leukemia with T-cell large granular lymphocytic leukemia

T-cell large granular lymphocyte leukemia (T-LGL) also known as T-cell chronic lymphocytic leukemia is rare and comprises a small minority of all small lymphocytic leukemias. The concomitant presentation of T-LGL with acute myeloid leukemia (AML) has not been previously reported. We present an elderly gentleman with concomitant T-LGL and AML (non-M3) diagnosed by a combination of morphologic evaluation, immunophenotyping by flow cytometry, and T-cell gene rearrangement studies. The patient was managed with combination AML chemotherapy. He remains alive and well seven months after initial diagnosis. A brief review of literature is also presented.

Bronchus-associated B-cell lymphoma occurring after radiation and chemotherapy for head and neck Merkel cell carcinoma

Dear Editor, Majority of extranodal marginal zone lymphomas (MZL) are seen in the stomach in mucosa-associated lymphoid tissue (MALT) due to chronic gastritis following infection by Helicobacter pylori [1]. In contrast however, MZL lymphomas of the bronchial-associated lymphoid tissue (BALT) are exceedingly uncommon and are usually associated with autoimmune diseases or long-standing inflammatory conditions [1]. Rarely, MALT lymphomas have been described in the setting of immunosuppression [1, 2]. Similarly, skin tumors like Merkel cell carcinoma (MCC) have also been seen after therapeutic immunosuppression post solid organ transplantation [3]. We report on a patient who developed BALT lymphoma (MZL of the lung) following treatment of MCC by a combination of chemotherapy and radiation therapy to the head and neck. Such an association has not been reported previously. A 79-year-old woman presented with a suspicious right eyebrow lesion, and examination revealed a palpable node in the area of the right parotid gland. The right eyebrow lesion was excised and revealed a MCC. A superficial parotidectomy performed to remove the palpable lymph node contained metastatic MCC. Chest radiographs, computed tomographic (CT) scans, and an indium-111-labeled octreotide scan failed to reveal any abnormality. She was treated with 50 Gy of adjuvant radiation over 5 weeks to the face and right side of the neck in combination with six cycles of chemotherapy (carboplatin and etoposide) given every 3 weeks. She was in remission for 5 years, when she presented with sudden onset of a tender swelling on the right neck. An ultrasound demonstrated a thick-walled 3.3-cm mass, and subsequent chest radiographs demonstrated a new opacity in the left lung. She was initiated on antibiotics that led to the resolution of the right neck mass only. Multiple bilateral pulmonary nodules were noted (Fig. 1a–d) on repeat scans; however, there was no adenopathy. Transthoracic needle biopsies revealed a marked proliferation of lymphocytes expanding the pulmonary parenchyma (Fig. 1e and f). The cells were medium-sized with round nuclei. Whereas some cells had a narrow rim of cytoplasm, others were plasmacytoid and contained more abundant cytoplasm and eccentrically placed nuclei. The cells were diffusely and strongly positive for B-cell markers CD20 (Fig. 1g), CD10, and CD43, and negative for CD5 and bcl-2. Background sheets of plasma cells showing cytoplasmic IgM kappa light chain restriction were present. These findings were consistent with extranodal marginal zone lymphoma of the BALT type with plasmacytic differentiation. A comprehensive bone marrow examination including flow cytometry analysis was negative for involvement by lymphoma. Ann Hematol (2006) 85:815–816 DOI 10.1007/s00277-006-0173-6

Cholecystocolonic fistula with impacted sigmoid gallstone mimicking malignant obstruction of the sigmoid colon

TO THE EDITOR: An 85-year-old woman presented to the ER with sudden worsening of a 4-day history of abdominal distention, progressive lower abdominal pain, constipation and non-bilious emesis. A CT scan was ordered to evaluate for intestinal obstruction and revealed impacted curvilinear 3.5 /2.9 cm opacification in the sigmoid colon (Figure 1A). Significant gallbladder wall thickening with surrounding edema and close approximation of the gallbladder to the right colon was also noted. A working diagnosis of acute abdomen due to sigmoid obstruction secondary to a stenosing mass lesion was made. The patient underwent a Gastrografin enema which showed a fistula between the hepatic flexure of the colon and the gallbladder and an obstructing lesion in the left colon (Figure 1B). The sigmoid density showed movement during the study, suggesting impacted stool. The patient underwent cholecystectomy and resection of the fistula and involved segment of the colon. The impacted mass was discovered to be a gallstone and was retrieved by milking it back into the proximal transverse colon at the time of division of the fistula. A second gallstone was retrieved from the gallbladder. The patient made an uneventful postoperative recovery. Gallstone obstruction of the large bowel is a rare condition. The gallstone should be at least 2 2.5 cm in diameter to cause obstruction [1]. Most patients with impacted colonic gallstones present with symptoms of large-bowel obstruction. The presentation is compounded by the fact that only a minority have a history of biliary symptoms in the recent past [1]. The classic signs on imaging studies include intestinal obstruction, pneumobilia, aberrantly located Figure 1. A. CT scan of the abdomen with a curvilinear obstructing mass in the sigmoid (white arrow ). B. Gastrografin enema demonstrated a fistula between the gallbladder and the colon (arrowheads ). The sigmoid mass was also confirmed.